WorldWideScience

Sample records for intradural retroclival chordoma

  1. Chordoma.

    Science.gov (United States)

    Casali, Paolo G; Stacchiotti, Silvia; Sangalli, Claudia; Olmi, Patrizia; Gronchi, Alessandro

    2007-07-01

    To review developments in chordoma treatment. Recent series with prolonged follow-up show that adequate margins are necessary for surgery to be curative. Safe margins are often difficult to obtain due to the anatomical sites of chordoma: sacrum, skull base and spine. Tumors in these sites are problematic for radiation therapy as well, and this adds to the need for high doses. Hadrons have therefore been used in addition to, or instead of, photons. New photon beam techniques, e.g. intensity modulated radiation therapy and stereotactic procedures, have recently been evaluated. Although less available than photons, hadrons possess certain advantages. While chemotherapy is poorly active, recent interest has focused on molecular-targeted agents. Imatinib was shown to be active, providing mainly nondimensional tissue responses in a significant proportion of patients, which may improve symptoms and progression-free interval. Epidermal growth factor receptor targeting, anti-angiogenics, and the combination of targeted agents with chemotherapy and radiation therapy are also under scrutiny. Two major issues about local treatment remain unresolved: when to complement surgery with radiation therapy, and how best to deliver high doses of radiation therapy to the tumor tissue. Regarding systemic treatment, there is ongoing research into how to exploit molecular-targeted therapies.

  2. Retroclival collections associated with abusive head trauma in children

    Energy Technology Data Exchange (ETDEWEB)

    Silvera, V.M.; Danehy, Amy R.; Carducci, Chiara; Grant, P.E.; Kleinman, Paul K. [Harvard Medical School, Department of Radiology, Boston Children' s Hospital, Boston, MA (United States); Newton, Alice W. [Harvard Medical School, Child Protection Program, Department of Pediatrics, Massachusetts General Hospital, Boston, MA (United States); Stamoulis, Catherine [Harvard Medical School, Department of Radiology, Boston Children' s Hospital, Boston, MA (United States); Harvard Medical School, Department of Neurology, Boston Children' s Hospital, Boston, MA (United States); Wilson, Celeste R. [Harvard Medical School, Division of General Pediatrics, Department of Medicine, Boston Children' s Hospital, Boston, MA (United States)

    2014-12-15

    Retroclival collections are rare lesions reported almost exclusively in children and strongly associated with trauma. We examine the incidence and imaging characteristics of retroclival collections in young children with abusive head trauma. We conducted a database search to identify children with abusive head trauma ≤3 years of age with brain imaging performed between 2007 and 2013. Clinical data and brain images of 65 children were analyzed. Retroclival collections were identified in 21 of 65 (32%) children. Ten (48%) were subdural, 3 (14%) epidural, 2 (10%) both, and 6 (28%) indeterminate. Only 8 of 21 retroclival collections were identifiable on CT and most were low or intermediate in attenuation. Eighteen of 21 retroclival collections were identifiable on MRI: 3 followed cerebral spinal fluid in signal intensity and 15 were bloody/proteinaceous. Additionally, 2 retroclival collections demonstrated a fluid-fluid level and 2 enhanced in the 5 children who received contrast material. Sagittal T1-weighted images, sagittal fluid-sensitive sequences, and axial FLAIR (fluid-attenuated inversion recovery) images showed the retroclival collections best. Retroclival collections were significantly correlated with supratentorial and posterior fossa subdural hematomas and were not statistically correlated with skull fracture or parenchymal brain injury. Retroclival collections, previously considered rare lesions strongly associated with accidental injury, were commonly identified in this cohort of children with abusive head trauma, suggesting that retroclival collections are an important component of the imaging spectrum in abusive head trauma. Retroclival collections were better demonstrated on MRI than CT, were commonly identified in conjunction with intracranial subdural hematomas, and were not significantly correlated with the severity of brain injury or with skull fractures. (orig.)

  3. Retroclival collections associated with abusive head trauma in children

    International Nuclear Information System (INIS)

    Silvera, V.M.; Danehy, Amy R.; Carducci, Chiara; Grant, P.E.; Kleinman, Paul K.; Newton, Alice W.; Stamoulis, Catherine; Wilson, Celeste R.

    2014-01-01

    Retroclival collections are rare lesions reported almost exclusively in children and strongly associated with trauma. We examine the incidence and imaging characteristics of retroclival collections in young children with abusive head trauma. We conducted a database search to identify children with abusive head trauma ≤3 years of age with brain imaging performed between 2007 and 2013. Clinical data and brain images of 65 children were analyzed. Retroclival collections were identified in 21 of 65 (32%) children. Ten (48%) were subdural, 3 (14%) epidural, 2 (10%) both, and 6 (28%) indeterminate. Only 8 of 21 retroclival collections were identifiable on CT and most were low or intermediate in attenuation. Eighteen of 21 retroclival collections were identifiable on MRI: 3 followed cerebral spinal fluid in signal intensity and 15 were bloody/proteinaceous. Additionally, 2 retroclival collections demonstrated a fluid-fluid level and 2 enhanced in the 5 children who received contrast material. Sagittal T1-weighted images, sagittal fluid-sensitive sequences, and axial FLAIR (fluid-attenuated inversion recovery) images showed the retroclival collections best. Retroclival collections were significantly correlated with supratentorial and posterior fossa subdural hematomas and were not statistically correlated with skull fracture or parenchymal brain injury. Retroclival collections, previously considered rare lesions strongly associated with accidental injury, were commonly identified in this cohort of children with abusive head trauma, suggesting that retroclival collections are an important component of the imaging spectrum in abusive head trauma. Retroclival collections were better demonstrated on MRI than CT, were commonly identified in conjunction with intracranial subdural hematomas, and were not significantly correlated with the severity of brain injury or with skull fractures. (orig.)

  4. Traumatic epidural retroclival hematoma with odontoid fracture and cardiorespiratory arrest.

    Science.gov (United States)

    Pérez-Bovet, J; Garcia-Armengol, R; Martín Ferrer, S

    2013-12-01

    Case report. To describe a case or traumatic retroclival hematoma with features not previously reported. Single center. Description of a case, in the context of relevant literature on the subject. Traumatic retroclival hematomas are a rare, typically pediatric, entity. Only four cases of epidural hematoma in adult patients have been reported. We describe an additional case, the first with a fatal course in the acute setting. It is also the first retroclival hematoma associated to an odontoid base fracture. Retroclival hematomas are a rare diagnosis, to be considered in pediatric patients with flexion-extension, high-energy injuries. Morphology is typically epidural. Brain stem and cranial nerve symptoms are typical. Treatment is usually conservative. Outcome is regarded as favorable, with partial recovery and neurologic sequelae. Adult cases are extremely rare. The case we describe adds new characteristics to the scarcity of cases.

  5. Cranial Chordoma: A New Preoperative Grading System.

    Science.gov (United States)

    Brito da Silva, Harley; Straus, David; Barber, Jason K; Rostomily, Robert C; Ferreira, Manuel; Sekhar, Laligam N

    2017-11-03

    Chordomas are rare but challenging neoplasms involving the skull base. A preoperative grading system will be useful to identify both areas for treatment and risk factors, and correlate to the degree of resection, complications, and recurrence. To propose a new grading system for cranial chordomas designed by the senior author. Its purpose is to enable comparison of different tumors with a similar pathology to clivus chordoma, and statistically correlate with postoperative outcomes. The numerical grading system included tumor size, site of the tumor, vascular encasement, intradural extension, brainstem invasion, and recurrence of the tumor either after surgery or radiotherapy with a range of 2 to 25 points; it was used in 42 patients with cranial chordoma. The grading system was correlated with number of operations for resection, degree of resection, number and type of complications, recurrence, and survival. We found 3 groups: low-risk 0 to 7 points, intermediate-risk 8 to 12 points, and high-risk ≥13 points in the grading system. The 3 groups were correlated with the following: extent of resection (partial, subtotal, or complete; P system itself correlated with the outcome (P = .005). The proposed chordoma grading system can help surgeons to predict the difficulty of the case and know which areas of the skull base will need attention to plan further therapy. © Congress of Neurological Surgeons 2017.

  6. Clinicopathological significance of p16, cyclin D1, Rb and MIB-1 levels in skull base chordoma and chondrosarcoma

    Directory of Open Access Journals (Sweden)

    Jun-qi Liu

    2015-09-01

    Full Text Available Objective: To investigate the expression of p16, cyclin D1, retinoblastoma tumor suppressor protein (Rb and MIB-1 in skull base chordoma and chondrosarcoma tissues, and to determine the clinicopathological significance of the above indexes in these diseases. Methods: A total of 100 skull base chordoma, 30 chondrosarcoma, and 20 normal cartilage tissue samples were analyzed by immunohistochemistry. The expression levels of p16, cyclinD1, Rb and MIB-1 proteins were assessed for potential correlation with the clinicopathological features. Results: As compared to normal cartilage specimen (control, there was decreased expression of p16, and increased expression of cyclin D1, Rb and MIB-1 proteins, in both skull base chordoma and chondrosarcoma specimens. MIB-1 LI levels were significantly increased in skull base chordoma specimens with negative expression of p16, and positive expression of cyclin D1 and Rb (P  0.05. However, p16 and MIB-1 levels correlated with the intradural invasion, and expression of p16, Rb and MIB-1 correlated with the number of tumor foci (P < 0.05. Further, the expression of p16 and MIB-1 appeared to correlate with the prognosis of patients with skull base chordoma. Conclusions: The abnormal expression of p16, cyclin D1 and Rb proteins might be associated with the tumorigenesis of skull base chordoma and chondrosarcoma. Keywords: p16, Cyclin D1, Rb, MIB-1, Skull base chordoma, Skull base chondrosarcoma

  7. Intradural cervical disc herniation: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Seung Eun; Lee, Sang Ho; Jo, Byung June; Yoon, Deug Hee [Wooridul Spine Hospital, Seoul (Korea, Republic of); Kim, Tae Hong [Inje University College of Medicine, Inje (Korea, Republic of)

    2005-07-15

    Intradural cervical disc herniation is an extremely rare condition and its pathogenesis is not certain. We experienced a case of intradural cervical disc herniation at the C4-5 level in a 56-year-old man. The preoperative sagittal T1- and T2- weighted images revealed an intradural iso-intensity lesion, with the spinal cord behind the posterior longitudinal ligament at the C4-5 disc level. The post-contrast T1-weighted image revealed a peripheral enhanced intradural lesion. We report here on a case of an intradural cervical disc herniation that was diagnosed by radiological examination, and we include a review of the related literature.

  8. Intradural disc herniation: how I do it.

    Science.gov (United States)

    Rodrigo, Víctor; Claramonte, Marta; Martín, Mónica; Calatayud, Juan B

    2018-03-12

    Intradural disc herniation is a rare phenomenon in spine surgery. Diagnosis is difficult despite current neuroradiologic imaging techniques. We present a case of a 59-year-old man with lumbar and radicular pain and a recurrent lumbar herniation. A laminectomy was performed after no clear disc herniation in the epidural space was found and an intradural mass was palpable. A durotomy showed an intradural disc fragment that was removed, followed by an arthrodesis. Only intraoperative findings lead to a definitive diagnosis for intradural herniation. A durotomy needs to be performed. In this case, an arthrodesis was necessary to avoid complications of segmental instability.

  9. Imatinib mesylate in chordoma.

    Science.gov (United States)

    Casali, Paolo G; Messina, Antonella; Stacchiotti, Silvia; Tamborini, Elena; Crippa, Flavio; Gronchi, Alessandro; Orlandi, Rosaria; Ripamonti, Carla; Spreafico, Carlo; Bertieri, Raffaello; Bertulli, Rossella; Colecchia, Maurizio; Fumagalli, Elena; Greco, Angela; Grosso, Federica; Olmi, Patrizia; Pierotti, Marco A; Pilotti, Silvana

    2004-11-01

    To the authors' knowledge, no effective medical therapy currently is available for advanced chordoma. Imatinib mesylate is a tyrosine kinase inhibitor targeting platelet-derived growth factor receptor-beta (PDGFRB), BCR-ABL, and KIT. Six patients with advanced chordoma were treated with imatinib mesylate at a dose of 800 mg daily. In all patients, the tumor was found to be positive for PDGFRB, and in four patients PDGFRB was shown to be phosphorylated/expressed. After a treatment period of > or = 1 year, overt tumor liquefaction was evident on computed tomography (CT) scan in the first patient. In previous months, a decrease in contrast enhancement on magnetic resonance imaging (MRI) and a decrease in glucose uptake on positron emission tomography (PET) were detected. Similar signs on MRI and PET were observed in subsequent patients, who had a shorter treatment period. One of these patients initially was removed from therapy and then was readmitted to therapy because of difficulties with regard to tumor response assessment; 1 month after the reinitiation of therapy, an overt decrease in tumor density was visible on CT scan in this patient. In four of five symptomatic patients, a subjective improvement was observed early in the course of treatment. The first patient died after 17 months, with a sizeable, mostly liquefied mass. Another patient died early, apparently of unrelated causes. The remaining patients were on therapy at the time of last follow-up. Imatinib mesylate has been found to have antitumor activity in patients with chordoma. This activity might be mediated by inactivation of PDGFRB. Tumor response manifests through patterns that are similar to those observed in patients with gastrointestinal stromal tumors who respond to molecular-targeted therapy, but evolves more slowly. The benefit to the patient entailed by this pattern of tumor response in chordoma needs to be elucidated, but may be limited in the presence of significant local disease.

  10. A historical recount of chordoma.

    Science.gov (United States)

    Sahyouni, Ronald; Goshtasbi, Khodayar; Mahmoodi, Amin; Chen, Jefferson W

    2018-02-02

    Chordoma, a rare bone tumor that occurs along the spine, has led scientists on a fascinating journey of discoveries. In this historical vignette, the authors track these discoveries in diagnosis and treatment, noting events and clinicians that played pivotal roles in our current understanding of chordoma. The study of chordoma begins in 1846 when Rudolf Virchow first observed its occurrence on a dorsum sellae; he coined the term "chordomata" 11 years later. The chordoma's origin was greatly disputed by members of the scientific community. Eventually, Müller's notochord hypothesis was accepted 36 years after its proposal. Chordomas were considered benign and slow growing until the early 1900s, when reported autopsy cases drew attention to their possible malignant nature. Between 1864 and 1919, the first-ever symptomatic descriptions of various forms of chordoma were reported, with the subsequent characterization of chordoma histology and the establishment of classification criteria shortly thereafter. The authors discuss the critical historical steps in diagnosis and treatment, as well as pioneering operations and treatment modalities, noting the physicians and cases responsible for advancing our understanding of chordoma.

  11. Clival chordoma in an infant.

    Directory of Open Access Journals (Sweden)

    Goel A

    1996-04-01

    Full Text Available An unusual case of clival chordoma seen in a 7 month 16 day old infant is presented. The literature on this subject, clinical course of such tumours and the management strategy in paediatric age group is reviewed.

  12. Vaccine Therapy for Unresectable Chordoma

    Science.gov (United States)

    In this phase II clinical trial, adult patients with inoperable chordoma who are scheduled to undergo radiation therapy will be randomly assigned to receive a yeast-based vaccine that targets a protein called brachyury or a placebo injection.

  13. Extra and intradural spinal Hemangioblastoma Hemangioblastoma espinal extra e intradural Hemangioblastoma espinhal extra e intradural

    Directory of Open Access Journals (Sweden)

    Marcelo Campos Moraes Amato

    2012-09-01

    Full Text Available Hemangioblastomas of the central nervous system (CNS are low-grade highly vascularized tumors that may be sporadic or associated with Von Hippel-Lindau disease. Extradural hemangioblastomas are uncommon and those located extra and intradurally are even rarer. This study uses an illustrative case and literature review to discuss the difficulties to consider the correct diagnosis and to select the best surgical approach. A 57 years-old white male patient presented with myelopathy and right C5 radiculopathy. The images showed a lobulated, hourglass shaped, highly enhanced extra/intradural lesion that occupied the spinal canal and widened the C4-C5 right intervertebral foramen. Total resection of the intradural lesion was achieved through a posterior approach, but the extradural part could only be partially removed. Complete improvement was observed after four months of follow-up and the residual tumor has been followed up clinically and radiologically. Even though the preoperative impression was of a spinal schwannoma, the histopathological examination revealed grade I hemangioblastoma as per WHO. Despite their rarity, current complementary exams allow considering the diagnosis of hemangioblastoma preoperatively. That is essential to a better surgical planning in view of the particular surgical features of this lesion.Hemangioblastomas del sistema nervioso central (SNC son tumores altamente vascularizados, de grado bajo, que pueden ser esporádicos o vinculados a la enfermedad de Von Hippel-Lindau. Hemangioblastomas extradurales no son comunes, y aquellos localizados extra e intraduralmente son aún más raros. Este estudio usa un caso ilustrativo y la revisión de la literatura para analizar las dificultades cuanto a considerar el diagnóstico correcto y para seleccionar el mejor abordaje quirúrgico. Un paciente, hombre blanco de 57 años de edad, presentaba mielopatía con radiculopatía C5 derecha. Las imágenes mostraban lesión extra/intradural

  14. Chordoma | Fitchart | South African Medical Journal

    African Journals Online (AJOL)

    The history, origin, pathology, diagnosis, treatment and prognosis of chordoma involving the axial skeleton are reviewed, and the clinical histories of 3 cases of chordoma are given, noting some remarkable features, along with one case misdiagnosed as chordoma on the radiological appearance of the sacrum and treated ...

  15. The driver landscape of sporadic chordoma.

    Science.gov (United States)

    Tarpey, Patrick S; Behjati, Sam; Young, Matthew D; Martincorena, Inigo; Alexandrov, Ludmil B; Farndon, Sarah J; Guzzo, Charlotte; Hardy, Claire; Latimer, Calli; Butler, Adam P; Teague, Jon W; Shlien, Adam; Futreal, P Andrew; Shah, Sohrab; Bashashati, Ali; Jamshidi, Farzad; Nielsen, Torsten O; Huntsman, David; Baumhoer, Daniel; Brandner, Sebastian; Wunder, Jay; Dickson, Brendan; Cogswell, Patricia; Sommer, Josh; Phillips, Joanna J; Amary, M Fernanda; Tirabosco, Roberto; Pillay, Nischalan; Yip, Stephen; Stratton, Michael R; Flanagan, Adrienne M; Campbell, Peter J

    2017-10-12

    Chordoma is a malignant, often incurable bone tumour showing notochordal differentiation. Here, we defined the somatic driver landscape of 104 cases of sporadic chordoma. We reveal somatic duplications of the notochordal transcription factor brachyury (T) in up to 27% of cases. These variants recapitulate the rearrangement architecture of the pathogenic germline duplications of T that underlie familial chordoma. In addition, we find potentially clinically actionable PI3K signalling mutations in 16% of cases. Intriguingly, one of the most frequently altered genes, mutated exclusively by inactivating mutation, was LYST (10%), which may represent a novel cancer gene in chordoma.Chordoma is a rare often incurable malignant bone tumour. Here, the authors investigate driver mutations of sporadic chordoma in 104 cases, revealing duplications in notochordal transcription factor brachyury (T), PI3K signalling mutations, and mutations in LYST, a potential novel cancer gene in chordoma.

  16. Primary Intradural Hemangiopericytoma With Intramedullary Invasion

    Directory of Open Access Journals (Sweden)

    Chiang-Wei Chou

    2009-10-01

    Full Text Available Hemangiopericytoma (HPC is a rare tumor of the central nervous system and is usually found intracranially. Intraspinal HPCs are very rare and mostly involve the extradural bony structures. Primary intradural HPC has only been reported in 10 cases, all of which occurred in the extramedullary region. Intramedullary invasion has never been reported. Here, we describe a case of primary intradural HPC of the thoracic spine that presented initially with paresthesia and paraplegia of both legs. Magnetic resonance imaging of the thoracic spine showed an intradural dumbbell-shaped tumor at the T10 level. The initial impression was neurogenic tumor, meningioma, or metastasis. During operation, the tumor was found to have obvious intramedullary invasion. Gross-total removal was done, and the patient's neurological function improved; there was no recurrence at the 3-year follow-up. There is no consensus as to what constitutes the optimal treatment of HPC, but most neurosurgeons will advocate gross-total resection. A comparative analysis between intradural and extradural HPCs showed a higher chance of gross-total resection for intradural HPCs, while the recurrence rates showed no difference. The role of adjuvant radiotherapy remains uncertain. Due to the high risk of recurrence and metastasis of HPCs, close follow-up for a long period is mandatory. [J Chin Med Assoc 2009;72(10:536–541

  17. CASE REPORT Lumbar vertebra chordoma

    African Journals Online (AJOL)

    vertebral column tumours. Introduction. Spinal chordomas mostly present in association with the clivus or sacrum. We report a case where it was overlooked in the differential diagnosis of vertebral col- umn tumours because it presented in the lumbar spine. Case. A 66-year-old male patient presented with a longstand-.

  18. Intradural lumbar disc herniation with intradural gas: report of three cases

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Seung Eun; Lee, Sang Ho; Jo, Byung June [Wooridul Spine Hospital, Seoul (Korea, Republic of); Kim, Tae Hong [Inje University College of Medicine, Seoul (Korea, Republic of)

    2005-12-15

    This paper reports on three cases of an intradural lumbar disc herniation (IDLDH) that were diagnosed by a radiological examination. In all cases, an intradural vacuum (IDV) was detected on the CT scans, and the IDLDH showed iso- or lower signal intensity on the T2-weighted images. Enhanced MRI of one case revealed a small amount of air, but this was without enhancement. All the cases showed definite IDV on the CT scans, and this was an important clue for diagnosing IDLDH.

  19. Intradural squamous cell carcinoma in the sacrum

    Directory of Open Access Journals (Sweden)

    Fujisawa Kozo

    2009-02-01

    Full Text Available Abstract Background Leptomeningeal carcinomatosis occurs in patients with cancer at the rate of approximately 5%; it develops particularly in patients with breast cancer, lung cancer, melanoma, leukemia, or malignant lymphoma. We describe a rare case of leptomeningeal carcinomatosis in which spinal intradural squamous cell carcinoma with no lesions in the cerebral meninges and leptomeninx, was the primary lesion. Methods A 64-year-old man complained of sacral pain. Although the patient was treated with analgesics, epidural block and nerve root block, sacral pain persisted. Since acute urinary retention occurred, he was operated on. The patient was diagnosed as having an intradural squamous cell carcinoma of unknown origin. Results Since the patient presented with a slightly decreased level of consciousness 2 months after surgery, he was subjected to MRI scanning of the brain and spinal cord, which revealed disseminated lesions in the medulla oblongata. The patient died of pneumonia and sepsis caused by methicillin-resistant Staphylococcus aureus 5 months after surgery. Conclusion We report the first case of a patient with intradural squamous cell carcinoma with unknown origin that developed independently in the sacrum.

  20. Chordoma: The Quest for Better Treatment Options.

    Science.gov (United States)

    Heery, Christopher R

    2016-01-01

    Chordoma is an extremely rare cancer, with an incidence of about one case per million persons per year in the USA and Europe (about 300 and 450 cases per year, respectively). The estimated median overall survival of patients with chordoma is approximately 6-7 years, yielding a rough estimate of chordoma prevalence at about 2000 in the USA and 3000 in Europe. Primary tumor develops along the axial spine between the clivus and sacrum and develops from the residual embryonic notochord. Brachyury (T), a transcription factor required for normal embryonic development, is expressed in the notochord and overexpressed in almost all cases of chordoma. The primary treatment for chordoma is surgical excision with wide local margins, when possible. Radiotherapy also plays a significant role in the adjuvant setting and when surgery is not possible. Unfortunately, in the advanced and/or metastatic setting, where the role of surgery and/or radiation is less clear, treatment options are very limited. To date, there have been no randomized, controlled trials in chordoma that have resulted in defined agents of clinical benefit for systemic treatment. This review briefly describes the natural history and initial treatment of chordoma and focuses on treatment options for advanced disease and potential avenues of research that may lead to improved treatment options in the future.

  1. Chordoma with postoperative subcutaneous implantation and meningeal dissemination: MRI

    International Nuclear Information System (INIS)

    Kinoshita, T.; Okudera, T.; Shimosegawa, E.; Hatazawa, J.; Yoshida, Y.; Yasui, N.; Ogawa, T.

    2001-01-01

    Chordomas are histologically benign tumours which are locally invasive. We present an unusual case of recurrent chordoma with subcutaneous implantation and widespread meningeal dissemination after surgery. Contrast-enhanced MRI was useful for determining the extent of the tumour. (orig.)

  2. Intracranial metastasis from a sacrococcygeal chordoma. Case report.

    LENUS (Irish Health Repository)

    Kamel, Mahmoud Hamdy

    2012-02-03

    Chordoma is a locally invasive tumor of low metastatic potential. Only six cases of chordoma that metastasized to the brain are found in the English literature. Most of these lesions were clinically silent and all were associated with extraneural metastases. The authors report a case of symptomatic brain metastasis from a sacrococcygeal chordoma in the absence of other metastases. The incidence, sites, and factors predictive of chordoma metastasis are discussed.

  3. A retrorectal tumor: Presacral chordoma

    Directory of Open Access Journals (Sweden)

    Suresh Birur Parmeshwarappa

    2013-01-01

    Full Text Available The retrorectal space contains multiple embryologic remnants derived from various tissues and tumors that develop in this space are both grossly and histologically heterogeneous. Most lesions are benign, but malignant neoplasms are not uncommon. Malignancy is more common in the pediatric population than in adults and solid lesions are more likely to be malignant than are cystic lesions. Here, 50-year-old male presented bleeding with mass per rectum and lower abdominal pain. The computed tomography scan showed retrorectal mass with destruction of the sacrum and excision of the presacral mass with removal of involved sacrum from third sacral vertebra to tip of coccyx. Finally, histopathology was chordoma of sacrum. Patient had received adjuvant external beam radiotherapy.

  4. Novel targeted therapies in chordoma: an update.

    Science.gov (United States)

    Di Maio, Salvatore; Yip, Stephen; Al Zhrani, Gmaan A; Alotaibi, Fahad E; Al Turki, Abdulrahman; Kong, Esther; Rostomily, Robert C

    2015-01-01

    Chordomas are rare, locally aggressive skull base neoplasms known for local recurrence and not-infrequent treatment failure. Current evidence supports the role of maximal safe surgical resection. In addition to open skull-base approaches, the endoscopic endonasal approach to clival chordomas has been reported with favorable albeit early results. Adjuvant radiation is prescribed following complete resection, alternatively for gross residual disease or at the time of recurrence. The modalities of adjuvant radiation therapy reported vary widely and include proton-beam, carbon-ion, fractionated photon radiotherapy, and photon and gamma-knife radiosurgery. As of now, no direct comparison is available, and high-level evidence demonstrating superiority of one modality over another is lacking. While systemic therapies have yet to form part of any first-line therapy for chordomas, a number of targeted agents have been evaluated to date that inhibit specific molecules and their respective pathways known to be implicated in chordomas. These include EGFR (erlotinib, gefitinib, lapatinib), PDGFR (imatinib), mTOR (rapamycin), and VEGF (bevacizumab). This article provides an update of the current multimodality treatment of cranial base chordomas, with an emphasis on how current understanding of molecular pathogenesis provides a framework for the development of novel targeted approaches.

  5. Chordoma

    African Journals Online (AJOL)

    dysphagia. X-ray films may reveal a lobulated soft tissue mass projecting into the vault of the nasopharynx. There may be erosion of the body of the sphenoid, that is, de- struction of the clivus. Inside the skull, there may be in- .... but also to the vigorous commitment of all to total phy- sical, mental and economic rehabilitation.

  6. Combined PDGFR and HDAC Inhibition Overcomes PTEN Disruption in Chordoma.

    Directory of Open Access Journals (Sweden)

    Dae-Hee Lee

    Full Text Available The majority of chordomas show activation of the platelet-derived growth factor receptor (PDGFR. Based on in vitro intertumoral variation in response to recombinant PDGF protein and PDGFR inhibition, and variable tumor response to imatinib, we hypothesized that chordomas resistant to PDGFR inhibition may possess downstream activation of the pathway.Molecular profiling was performed on 23 consecutive chordoma primary tissue specimens. Primary cultures established from 20 of the 23 specimens, and chordoma cell lines, UCH-1 and UCH-2, were used for in vitro experiments.Loss of heterozygosity (LOH at the phosphatase and tensin homolog (PTEN locus was observed in 6 specimens (26%. PTEN disruption statistically correlated with increased Ki-67 proliferation index, an established marker of poor outcome for chordoma. Compared to wild type, PTEN deficient chordomas displayed increased proliferative rate, and responded less favorably to PDGFR inhibition. PTEN gene restoration abrogated this growth advantage. Chordomas are characterized by intratumoral hypoxia and local invasion, and histone deacetylase (HDAC inhibitors are capable of attenuating both hypoxic signaling and cell migration. The combination of PDGFR and HDAC inhibition effectively disrupted growth and invasion of PTEN deficient chordoma cells.Loss of heterozygosity of the PTEN gene seen in a subset of chordomas is associated with aggressive in vitro behavior and strongly correlates with increased Ki-67 proliferative index. Combined inhibition of PDGFR and HDAC attenuates proliferation and invasion in chordoma cells deficient for PTEN.

  7. Combined PDGFR and HDAC Inhibition Overcomes PTEN Disruption in Chordoma.

    Science.gov (United States)

    Lee, Dae-Hee; Zhang, Ying; Kassam, Amin B; Park, Myung-Jin; Gardner, Paul; Prevedello, Daniel; Henry, Stephanie; Horbinski, Craig; Beumer, Jan H; Tawbi, Hussein; Williams, Brian J; Shaffrey, Mark E; Egorin, Merrill J; Abounader, Roger; Park, Deric M

    2015-01-01

    The majority of chordomas show activation of the platelet-derived growth factor receptor (PDGFR). Based on in vitro intertumoral variation in response to recombinant PDGF protein and PDGFR inhibition, and variable tumor response to imatinib, we hypothesized that chordomas resistant to PDGFR inhibition may possess downstream activation of the pathway. Molecular profiling was performed on 23 consecutive chordoma primary tissue specimens. Primary cultures established from 20 of the 23 specimens, and chordoma cell lines, UCH-1 and UCH-2, were used for in vitro experiments. Loss of heterozygosity (LOH) at the phosphatase and tensin homolog (PTEN) locus was observed in 6 specimens (26%). PTEN disruption statistically correlated with increased Ki-67 proliferation index, an established marker of poor outcome for chordoma. Compared to wild type, PTEN deficient chordomas displayed increased proliferative rate, and responded less favorably to PDGFR inhibition. PTEN gene restoration abrogated this growth advantage. Chordomas are characterized by intratumoral hypoxia and local invasion, and histone deacetylase (HDAC) inhibitors are capable of attenuating both hypoxic signaling and cell migration. The combination of PDGFR and HDAC inhibition effectively disrupted growth and invasion of PTEN deficient chordoma cells. Loss of heterozygosity of the PTEN gene seen in a subset of chordomas is associated with aggressive in vitro behavior and strongly correlates with increased Ki-67 proliferative index. Combined inhibition of PDGFR and HDAC attenuates proliferation and invasion in chordoma cells deficient for PTEN.

  8. Intracranial chordomas: para and intrasellar localization - report of two cases

    International Nuclear Information System (INIS)

    Cerioni Junior, M.; Romero, P.C.; Peres, A.J.; Cecilio, S.; Botelho, R.V.; Caldas, J.G.; Settanni, F.

    1992-01-01

    Two patients with chordomas are reported. One 39-year-old woman with intrasellar chordoma which suffered of galactorrhea, amenorrhea and bi temporal hemianopsia. Another patient, a 50-year-old woman with left parasellar chordoma with proptosis, progressive blindness and left sided facial pain. Clinical and radiological findings, including CT-scan and MRI, are discussed. The MRI in one patient showed a persistent tumor. (author)

  9. Combined PDGFR and HDAC Inhibition Overcomes PTEN Disruption in Chordoma

    OpenAIRE

    Lee, Dae-Hee; Zhang, Ying; Kassam, Amin B.; Park, Myung-Jin; Gardner, Paul; Prevedello, Daniel; Henry, Stephanie; Horbinski, Craig; Beumer, Jan H.; Tawbi, Hussein; Williams, Brian J.; Shaffrey, Mark E.; Egorin, Merrill J.; Abounader, Roger; Park, Deric M.

    2015-01-01

    Background The majority of chordomas show activation of the platelet-derived growth factor receptor (PDGFR). Based on in vitro intertumoral variation in response to recombinant PDGF protein and PDGFR inhibition, and variable tumor response to imatinib, we hypothesized that chordomas resistant to PDGFR inhibition may possess downstream activation of the pathway. Methods Molecular profiling was performed on 23 consecutive chordoma primary tissue specimens. Primary cultures established from 20 o...

  10. Metastatic Chordoma: A Diagnostic Challenge on Fine Needle Aspiration

    Directory of Open Access Journals (Sweden)

    Ghassan Tranesh

    2016-01-01

    Full Text Available Chordomas are primary low grade malignant tumors of bone that usually arise within both ends of axial skeleton. The Notochord is a midline, ectoderm-derived structure that defines the phylum of chordates. Chordomas may pose difficult diagnostic challenges when encountered in secondary locations, such as lungs or other parenchymatous organs. We report the cytologic findings of a metastatic chordoma sampled through CT-scan guided fine needle aspiration (FNA of lower lobe lung nodule in a 54-year-old man diagnosed with recurrent chordoma involving the lumber spine and paraspinal region.

  11. Clival chordoma manifesting as nasal bleeding. A case report

    Energy Technology Data Exchange (ETDEWEB)

    Kitai, Ryuhei; Yoshida, Kazuhiko; Kubota, Toshihiko; Sato, Kazufumi; Handa, Yuji; Kasahara, Kazuma [University of Fukui, Department of Neurosurgery, Fukui (Japan); Nakajima, Hirofumi [Tsuruga Municipal Hospital, Department of Neurosurgery, Fukui (Japan)

    2005-05-01

    Chordoma is a rare cartilaginous tumor, for which bleeding presentation is unusual. We report a case of rare hemorrhaged clival chordoma, which was diagnosed correctly by magnetic resonance imaging. A 32-year-old man presented with nasal bleeding. The tumor was totally removed via a trans-sphenoidal approach, from which the surgical specimen confirmed chordoma. Epistaxis seemed to be caused by the spreading of the intratumoral hemorrhage into the sphenoid sinus. This case demonstrates the importance of an exact differential diagnostic evaluation, including chordoma, by use of modern imaging techniques for nasal bleeding. (orig.)

  12. Clival chordoma manifesting as nasal bleeding. A case report

    International Nuclear Information System (INIS)

    Kitai, Ryuhei; Yoshida, Kazuhiko; Kubota, Toshihiko; Sato, Kazufumi; Handa, Yuji; Kasahara, Kazuma; Nakajima, Hirofumi

    2005-01-01

    Chordoma is a rare cartilaginous tumor, for which bleeding presentation is unusual. We report a case of rare hemorrhaged clival chordoma, which was diagnosed correctly by magnetic resonance imaging. A 32-year-old man presented with nasal bleeding. The tumor was totally removed via a trans-sphenoidal approach, from which the surgical specimen confirmed chordoma. Epistaxis seemed to be caused by the spreading of the intratumoral hemorrhage into the sphenoid sinus. This case demonstrates the importance of an exact differential diagnostic evaluation, including chordoma, by use of modern imaging techniques for nasal bleeding. (orig.)

  13. Novel targeted therapies in chordoma: an update

    Directory of Open Access Journals (Sweden)

    Di Maio S

    2015-05-01

    Full Text Available Salvatore Di Maio,1 Stephen Yip,2 Gmaan A Al Zhrani,3,4 Fahad E Alotaibi,3,4 Abdulrahman Al Turki,3,4 Esther Kong,2 Robert C Rostomily5 1Division of Neurosurgery, Jewish General Hospital, McGill University, Montreal, QC, 2Department of Pathology and Laboratory Medicine, Vancouver General Hospital, University of British Columbia, Vancouver, BC, Canada; 3National Neuroscience Institute, Department of Neurosurgery, King Fahad Medical City, Riyadh, Saudi Arabia; 4Department of Neurology and Neurosurgery, The Montreal Neurological Institute and Hospital, McGill University Health Centre, Montreal, QC, Canada; 5Department of Neurological Surgery, University of Washington, University of Washington Medical Center, Seattle, WA, USA Abstract: Chordomas are rare, locally aggressive skull base neoplasms known for local recurrence and not-infrequent treatment failure. Current evidence supports the role of maximal safe surgical resection. In addition to open skull-base approaches, the endoscopic endonasal approach to clival chordomas has been reported with favorable albeit early results. Adjuvant radiation is prescribed following complete resection, alternatively for gross residual disease or at the time of recurrence. The modalities of adjuvant radiation therapy reported vary widely and include proton-beam, carbon-ion, fractionated photon radiotherapy, and photon and gamma-knife radiosurgery. As of now, no direct comparison is available, and high-level evidence demonstrating superiority of one modality over another is lacking. While systemic therapies have yet to form part of any first-line therapy for chordomas, a number of targeted agents have been evaluated to date that inhibit specific molecules and their respective pathways known to be implicated in chordomas. These include EGFR (erlotinib, gefitinib, lapatinib, PDGFR (imatinib, mTOR (rapamycin, and VEGF (bevacizumab. This article provides an update of the current multimodality treatment of cranial base

  14. High-resolution Whole-Genome Analysis of Skull Base Chordomas Implicates FHIT Loss in Chordoma Pathogenesis

    Directory of Open Access Journals (Sweden)

    Roberto Jose Diaz

    2012-09-01

    Full Text Available Chordoma is a rare tumor arising in the sacrum, clivus, or vertebrae. It is often not completely resectable and shows a high incidence of recurrence and progression with shortened patient survival and impaired quality of life. Chemotherapeutic options are limited to investigational therapies at present. Therefore, adjuvant therapy for control of tumor recurrence and progression is of great interest, especially in skull base lesions where complete tumor resection is often not possible because of the proximity of cranial nerves. To understand the extent of genetic instability and associated chromosomal and gene losses or gains in skull base chordoma, we undertook whole-genome single-nucleotide polymorphism microarray analysis of flash frozen surgical chordoma specimens, 21 from the clivus and 1 from C1 to C2 vertebrae. We confirm the presence of a deletion at 9p involving CDKN2A, CDKN2B, and MTAP but at a much lower rate (22% than previously reported for sacral chordoma. At a similar frequency (21%, we found aneuploidy of chromosome 3. Tissue microarray immunohistochemistry demonstrated absent or reduced fragile histidine triad (FHIT protein expression in 98% of sacral chordomas and 67%of skull base chordomas. Our data suggest that chromosome 3 aneuploidy and epigenetic regulation of FHIT contribute to loss of the FHIT tumor suppressor in chordoma. The finding that FHIT is lost in a majority of chordomas provides new insight into chordoma pathogenesis and points to a potential new therapeutic target for this challenging neoplasm.

  15. Proton radiation therapy for clivus chordoma

    International Nuclear Information System (INIS)

    Yoshii, Yoshihiko; Tsunoda, Takashi; Hyodo, Akio; Nose, Tadao; Tsujii, Hirohiko; Tsuji, Hiroshi; Inada, Tetsuo; Maruhashi, Akira; Hayakawa, Yoshinori.

    1993-01-01

    A 57-year-old male with clival chordoma developed severe hoarseness, dysphagia, and dysphonia 1 month after a second removal of the tumor. Magnetic resonance imaging demonstrated a mass 10 cm in diameter in the region of the middle clivus enhanced inhomogeneously by gadolinium-diethylenetriaminepenta-acetic acid, and a defect in the skull base. There was evidence of compression of the anterior surface of the pons. He received proton irradiation employing a pair of parallel opposed lateral proton beams. The dose aimed at the tumor mass was 75.5 Gy, to the pharyngeal wall less than 38 Gy, and to the anterior portion of the pons less than 30 Gy. Time dose and fractionation factor was calculated at 148. Thirty-one months following treatment, he was free of clinical neurological sequelae. Proton therapy should be considered in treatment planning following initial surgical removal or for inoperable clivus chordoma. (author)

  16. Cervical chordoma managed with multidisciplinary surgical approach.

    Science.gov (United States)

    Ahsan, Farhan; Inglis, Tom; Allison, Robert; Inglis, Grahame S

    2011-05-01

    This paper describes the interdisciplinary management of a 62-year-old man who presented with a cervical chordoma of C2/3. This is a rare neoplasm of the axial skeleton which is usually treated surgically. This is technically challenging due to the surrounding anatomy and requirement for wide exposure. A number of surgical approaches have been described to access the clivus and upper cervical spine. This case involved both the Orthopaedic and Otolaryngology Head and Neck Surgery departments. Trotter's surgical technique was used to gain access for excision of the cervical chordoma and there was collaboration with an Orthopaedic Biotechnology Company in which a bio-model of the spine was created and a corpectomy cage specific to the patient developed. This approach allowed excellent visualisation of the tumour and the unique cage and plate achieved immediate stability and long term fusion. An interdisciplinary approach should be used in the management of upper cervical chordomas to facilitate tumour resection and reduce the potential for recurrence. © 2010 The Authors. ANZ Journal of Surgery © 2010 Royal Australasian College of Surgeons.

  17. Intradural hemangiopericytoma of the lumbar spine: a rare entity.

    Science.gov (United States)

    Fitzpatrick, D; Mahajan, J; Lewkowitz, M; Black, K; Setton, A; Woldenberg, R

    2009-01-01

    We report a case of a patient with an intradural hemangiopericytoma of the lumbar spine and the unusual MR angiography (MRA) and spinal angiography findings of arteriovenous shunting with spinal venous congestion. We highlight the concordance of the unusual MRA and angiographic findings and their relationship to combined endovascular and surgical therapy.

  18. Spinal cerebrospinal fluid seeding of a clival chordoma; A case report

    Energy Technology Data Exchange (ETDEWEB)

    Baek, Seung Hwan; Yu, In Kyu; Kim, Seong Min; Park, Ki Seok; Son, Hyun Jin [Eulji University Hospital, Daejeon (Korea, Republic of)

    2015-07-15

    Chordomas originate from remnants of the embryonic notochord and account for < 2% of all malignant bone tumors. Chordomas have a high rate of local recurrence. However, spinal cerebrospinal fluid (CSF) seeding of a chordoma is extremely rare. Here, we present a very rare case of clival chordoma with spinal seeding. Radiologists should consider spinal CSF seeding of a clival chordoma, particularly when accompanied by signs of dural perforation or caudal extension.

  19. Update on the Cytogenetics and Molecular Genetics of Chordoma

    Directory of Open Access Journals (Sweden)

    Larizza Lidia

    2005-02-01

    Full Text Available Abstract Chordoma is a rare mesenchymal tumour of complex biology for which only histologic and immunohistochemical criteria have been defined, but no biomarkers predicting the clinical outcome and response to treatment have yet been recognised. We herein review the interdisciplinary information achieved by epidemiologists, neurosurgeons and basic scientists on chordoma, usually a sporadic tumour, which also includes a small fraction of familial cases. Main focus is on the current knowledge of the genetic alterations which might pinpoint candidate genes and molecular mechanisms shared by sporadic and familiar chordomas. Due to the scarcity of the investigated tumour specimens and the multiple chromosome abnormalities found in tumours with aberrant karyotypes, conventional cytogenetics and Fluorescence In Situ Hybridization failed to detect recurrent chordoma-specific chromosomal rearrangements. Genome-wide approaches such as Comparative Genomic Hybridization (CGH are yet at an initial stage of application and should be implemented using BAC arrays either genome-wide or targeting selected genomic regions, disclosed by Loss of Heterozygosity (LOH studies. An LOH region was shown by a systematic study on a consistent number of chordomas to encompass 1p36, a genomic interval where a candidate gene was suggested to reside. Despite the rarity of multiplex families with chordoma impaired linkage studies, a chordoma locus could be mapped to chromosome 7q33 by positive lod score in three independent families. The role in chordomagenesis of the Tuberous Sclerosis Complex (TSC genes has been proved, but the extent of involvement of TSC1 and TSC2 oncosuppressors in chordoma remains to be assessed. In spite of the scarce knowledge on the genetics and molecular biology of chordoma, recent initiation of clinical trials using molecular-targeted therapy, should validate new molecular targets and predict the efficacy of a given therapy. Comparative genetic and

  20. Lumbar vertebra chordoma | Erlank | SA Journal of Radiology

    African Journals Online (AJOL)

    Abstract. Spinal chordomas in the lumbar region are rare and can easily be overlooked in the differential diagnosis of vertebral column tumours. South African Journal of Radiology Vol. 10 (3) 2006: pp. 37-38 ...

  1. Cardiac Metastasis from Clivus Chordoma: A Case Report.

    Science.gov (United States)

    Kim, Jun Won; Hong, Chang-Ki; Cha, Yoon Jin; Kim, Se Hoon; Suh, Chang-Ok; Lee, Kyu-Sung

    2018-02-14

    Chordomas are rare tumors showing locally aggressive nature and high rates of local recurrences. Distant metastases are infrequently reported and cardiac metastases are extremely rare. A 32-year-old woman had been diagnosed with a large chordoma involving the clivus. She received a total removal of the tumor and adjuvant intensity-modulated radiotherapy. Four and a half years after the surgery, she presented with multifocal lesions in the ventricles and the right atrium, although an open thoracotomy revealed chronic inflammation. Two years after finding the cardiac lesions, thoracoscopic biopsy of the chest wall lesion revealed metastatic chordoma. She received palliative imatinib for 2 weeks before she died of tumor progression. This case illustrates the metastatic potential of clivus chordoma and the heart can be a site of metastasis. Copyright © 2018. Published by Elsevier Inc.

  2. The effects of chemotherapeutic agents on differentiated chordoma cells.

    Science.gov (United States)

    Bayrak, Omer Faruk; Aydemir, Esra; Gulluoglu, Sukru; Sahin, Fikrettin; Sevli, Serhat; Yalvac, Mehmet Emir; Acar, Hasan; Ozen, Mustafa

    2011-12-01

    Chordoma is a rare type of malignant bone tumor and is known to arise from the remnants of the notochord. Resistance to chemotherapy makes the treatment of chordoma difficult; therefore, new approaches need to be developed to cure this disease. Differentiation therapy, using various differentiating agents, is attracting oncologists as a common therapeutic method to treat other tumors. Based on forcing cells to mature into other lineages, differentiation therapy might be an available method to treat chordomas in addition to conventional therapies. In this study a chordoma cell line, U-CH1, was exposed to several chemotherapeutic agents including vincristine, doxorubicin, cisplatin, etoposide, fludarabine, methotrexate, nilotinib, and imatinib mesylate under appropriate conditions. The first group of U-CH1 cells was exposed to drugs only and the second group of cells was exposed to the simultaneous treatment of 1 μM all-trans retinoic acid (ATRA) and chemotherapeutic agents in differentiation therapy. The efficacy of the differentiation method was assessed by measuring the viability of U-CH1 cells. Vincristine, doxorubicin, etoposide, cisplatin, and fludarabine, each at a concentration of 10 μM, decreased the number of chordoma cells when given alone down to 11%, 0%, 30%, 67%, and 3%, respectively. Etoposide and cisplatin, each at a concentration of 10 μM, reduced the percentage of viable chordoma cells in a more effective way when given with 1 μM ATRA simultaneously, reducing the number of viable cells to 14% and 9%, respectively. On the other hand, imatinib and nilotinib, each at a concentration of 3 μM, as well as 10 μM methotrexate, showed no decrease in the number of cancer cells. The results suggest that chordoma cells may be treated using the differentiation method in a more effective way than when they are treated with chemotherapeutic agents alone. This new approach may be an alternative method to conventional therapies in the treatment of chordoma.

  3. Surgical Consideration for Adolescents and Young Adults With Cervical Chordoma.

    Science.gov (United States)

    Zhong, Nanzhe; Yang, Xinghai; Yang, Jian; Meng, Tong; Yang, Cheng; Yan, Wangjun; Xiao, Jianru

    2017-05-15

    Retrospective study. The aim of this study was to compare the clinical outcomes between adolescent and young adult (AYA) patients and old adult patients with cervical chordoma who were treated surgically and present the surgical consideration for adolescents and young adults with cervical chordoma. With predominance in senior patients, chordoma is distinctively rare in AYAs. Because of the rarity of AYA chordoma, individual case report represents most of the literature on this disease entity on mobile spine and lack of long-term follow up, which leads to the paucity of clinical evidence for treatment planning and prognosis prediction. A retrospective study was conducted to investigate the prognosis of AYA patients with cervical chordoma who were treated surgically. We collected the clinical data of these patients and their older counterparts, and further compared the prognosis of the patients in different age groups. To estimate survival curves, Kaplan-Meier method was used, and significance was assessed using a log-rank test. Forty consecutive patients with chordoma of the cervical spine treated in our institution were included in the study. Two groups were identified according to age. Group 1 comprised children and adolescents (age ≤ 25 yrs; n = 9) and Group 2 comprised adults (age > 25 years; n = 31). In comparison, Group 1 was featured by significantly higher rate of recurrence and shorter overall survival, although no difference found in the surgical modality between two groups. There is a dismal prognosis in young patients with chordoma, and thus support the notion that as radical a total en bloc spondylectomy (TES) of the lesions as possible may benefit the overall survival of these young patients. Although the ensuing neurological deficits may be devastating, it will be worth sacrificing if the life expectancy of these young patients is prolonged. 4.

  4. Upper cervical spine chordoma of C2-C3.

    Science.gov (United States)

    Jiang, Liang; Liu, Zhong Jun; Liu, Xiao Guang; Ma, Qing Jun; Wei, Feng; Lv, Yang; Dang, Geng Ting

    2009-03-01

    Chordoma is a rare low-grade malignant neoplasm derived from the remnants of the embryonic notochord. This locally invasive neoplasm is subject to recurrence after treatment. The median survival time is estimated to be 6.3 years. Various treatment approaches have been attempted, including radical excision, radiotherapy and chemotherapy. Treatment outcome is significantly influenced by the size and site of the chordoma. Recently, Imatinib, a molecular-targeted agent, has been shown to have antitumor activity in chordoma. Proton radiotherapy, stereotactic radiotherapy and intensity-modulated radiotherapy have also been used. Surgical treatment is still the primary choice for chordoma. It has become more aggressive in recent years, evolving from intralesional or partial excision to en bloc resection. However, upper cervical localizations make such en bloc resection in most cases not possible. We present and discuss the therapeutic challenges of a young female with large retropharyngeal chordoma who presented to our institution after conventional photon beam radiotherapy. This C2/3 tumor was classified IB according to the Enneking classification. It distributed to layers A-D and sectors 1-6 according to the Weinstein Boriani Biagini Classification. The left vertebral artery (VA) was encapsulated and displaced. One stage intralesional extracapsular tumor excision and reconstruction was achieved by combined bilateral high anterior cervical approaches and posterior approach. No recurrence or metastasis was observed 3 years after the operation. She returned to her previous occupation as office worker.

  5. Response to imatinib plus sirolimus in advanced chordoma.

    Science.gov (United States)

    Stacchiotti, S; Marrari, A; Tamborini, E; Palassini, E; Virdis, E; Messina, A; Crippa, F; Morosi, C; Gronchi, A; Pilotti, S; Casali, P G

    2009-11-01

    Imatinib (IM) is active in advanced chordoma. The evidence of upstream and/or downstream mammalian target of rapamycin (mTOR) pathway activation prompted us to combine an mTOR inhibitor, sirolimus, to IM in IM-resistant advanced chordoma. Since July 2007, 10 progressive advanced chordoma patients with secondary resistance to IM, and biochemical and/or immunohistochemical evidence of upstream and/or downstream mTOR effector activation, started IM (400 mg/day) plus sirolimus (2 mg/day) on a named basis. The mean treatment duration was 9 months. Of nine patients assessable for response, at 3 months, we had one RECIST partial response (PR), seven stable disease (SD) and one progressive disease (PD). According to Choi criteria applied even to magnetic resonance imaging, we had seven PR (> or =10% decrease in size in four cases), one SD and one PD. Seven patients had a positron emission tomography response. The clinical benefit [RECIST complete response + PR + SD > or =6 months] was 89%. Pretreatment mTOR effectors analysis carried out in nine cases was positive in all patients (AKT activation in six patients, S6Sp6 expression/activation in seven). Post-treatment biopsy in one responsive patient confirmed S6 switch off. In addition to PDGFRB, mTOR pathway can be activated in chordomas and the combination of IM plus rapalogs may be effective in IM-resistant chordomas.

  6. The Specific Sagittal Magnetic Resonance Imaging of Intradural Extra-Arachnoid Lumbar Disc Herniation

    Directory of Open Access Journals (Sweden)

    Tatsuro Sasaji

    2012-01-01

    Full Text Available Intradural extra-arachnoid lumbar disc herniation is a rare disease. Few MRI findings have been reported. We experienced an intradural extra-arachnoid lumbar disc herniation. We reviewed the preoperative MRI findings. Lumbar spine T2-weighted sagittal MRI showed that one line of the ventral dura was divided into two by a disc herniation. We speculated that the two lines comprised the dura and arachnoid and that a disc herniation existed between them. We believe that division of the ventral dural line on T2-weighted sagittal images is a characteristic finding of intradural extra-arachnoid lumbar disc herniation. The division of ventral dural line seemed to be a “Y,” and, thus, we called it the “Y sign.” The “Y sign” may be useful for diagnosing intradural extra-arachnoid lumbar disc herniation.

  7. The Specific Sagittal Magnetic Resonance Imaging of Intradural Extra-Arachnoid Lumbar Disc Herniation

    Science.gov (United States)

    Sasaji, Tatsuro; Horaguchi, Kiyoshi; Yamada, Noboru; Iwai, Kazuo

    2012-01-01

    Intradural extra-arachnoid lumbar disc herniation is a rare disease. Few MRI findings have been reported. We experienced an intradural extra-arachnoid lumbar disc herniation. We reviewed the preoperative MRI findings. Lumbar spine T2-weighted sagittal MRI showed that one line of the ventral dura was divided into two by a disc herniation. We speculated that the two lines comprised the dura and arachnoid and that a disc herniation existed between them. We believe that division of the ventral dural line on T2-weighted sagittal images is a characteristic finding of intradural extra-arachnoid lumbar disc herniation. The division of ventral dural line seemed to be a “Y,” and, thus, we called it the “Y sign.” The “Y sign” may be useful for diagnosing intradural extra-arachnoid lumbar disc herniation. PMID:22431932

  8. Coexistence of intervertebral disc herniation with intradural schwannoma in a lumbar segment: a case report.

    Science.gov (United States)

    Pan, Jianjiang; Wang, Yue; Huang, Yazeng

    2016-04-18

    Lumbar intervertebral disc herniation and spinal tumor are major pathologies that may cause back pain and radiculopathy. Neurological symptoms resulting from disc herniation and intradural spinal tumor together, however, are very rare. We report a case of lumbar disc herniation which coexists with intradural schwannoma at the same spinal level in a 67-year-old man. The patient presented with persistent low back pain, sciatica, and weakness of the lower limbs. Contrast lumbar spine magnetic resonance (MR) imaging clearly delineated an intradural lesion and an extradural herniated disc at L3/4 level. Using a single posterior approach, both pathologies were addressed. Pathological studies confirmed the intradural lesion was schwannoma. The case report highlights a rare concomitance of two symptomatic pathologies in a lumbar spine, which deserves clinical attention. Complete history, careful physical examination, and investigative measures, such as contrast MR imaging, are helpful to establish throughout diagnoses.

  9. Cervical spine intradural-extramedullary hematoma presenting as ipsilateral hemiparesis

    Directory of Open Access Journals (Sweden)

    Chih Ming Lin

    2011-08-01

    Full Text Available A 75-year-old Taiwanese man suffered from acute onset of right-sided extremity weakness while talking to his neighbors. He was transferred to the hospital within three-hour time after symptom onset. Initial acute ischemic cerebral infarct was diagnosed based on his symptom and cerebral computed tomography. Thrombolytic therapy was held after his symptom improved promptly and could not excluded other etiology. Thorough history taking unraveled previous Chinese medicine clinic visit because of neck sore. However, he received limited improvement after several times of massage treatment. Magnetic resnance imaging (MRI of the cervical spine demonstrated hematoma compressing right side intradural-extramedullary space at the C2/C3 level. Through his clinical course, muscle weakness was the sole neurological finding with sparing of sensory defects. Given the close anatomy relationship between sensory and motor lamina distribution in the cervical spinal cord, our patient presented a rare manifestation. Cases of cervical spine intradural-extramedullary hematoma are not often seen and only sporadic in the documented literature. We wish, through the report of this article, to inform the first- line physicians with the following information. Among the elderly, neck sore is a common symptom. Over- stretching or overt local massage is not suggested due to relatively fragile musculature. In the clinical diagnosis and localization of lesion, cerebral or cervical spine lesion could mimic with each other and manifest hemiparesis as their first symptom. Meticulous history taking, neurological/ physical examination and pertinent laboratory work-up should be done before initiation of intravenous thrombolytic therapy as it could cause catastrophic consequences if not used properly.

  10. The Specific Sagittal Magnetic Resonance Imaging of Intradural Extra-Arachnoid Lumbar Disc Herniation

    OpenAIRE

    Sasaji, Tatsuro; Horaguchi, Kiyoshi; Yamada, Noboru; Iwai, Kazuo

    2012-01-01

    Intradural extra-arachnoid lumbar disc herniation is a rare disease. Few MRI findings have been reported. We experienced an intradural extra-arachnoid lumbar disc herniation. We reviewed the preoperative MRI findings. Lumbar spine T2-weighted sagittal MRI showed that one line of the ventral dura was divided into two by a disc herniation. We speculated that the two lines comprised the dura and arachnoid and that a disc herniation existed between them. We believe that division of the ventral du...

  11. Episodic cauda equina compression from an intradural lumbar herniated disc: a case of 'floppy disc'.

    Science.gov (United States)

    Nagaria, J; Chan, Cc; Kamel, Mh; McEvoy, L; Bolger, C

    2011-09-01

    Intradural disc herniation (IDDH) is a rare complication of intervertebral disc disease and comprises 0.26-0.30% of all herniated discs, with 92% of them located in the lumbar region (1). We present a case of IDDH that presented with intermittent symptoms and signs of cauda equina compression. We were unable to find in the literature, any previously described cases of intermittent cauda equina compression from a herniated intradural disc fragment leading to a "floppy disc syndrome". © JSCR.

  12. Sacral chordoma: a diagnosis not to be sat on?

    Science.gov (United States)

    Gibbins, Ruth; Evans, Guy; Grimer, Robert

    2007-01-01

    Sacral chordomas are rare, slow-growing tumours that are amenable to surgery, but unfortunately often diagnosed late. The aim of the study was to identify presenting symptoms, which may aid diagnosis and reduce the treatment time. Forty-four patients were identified with sacral chordoma between 1989 and 2006. Clinical and pathological records were reviewed retrospectively to elicit the symptoms recorded prior to diagnosis, duration of symptoms, surgical treatment, size of tumour and survival. Eleven patients were excluded, leaving 33 patients in the study group. Thirty-one patients had chordomas arising from the sacrum and two patients from the coccyx. The mean duration of symptoms prior to diagnosis was 120 weeks (2.3 years), with a median of length of 104 weeks (two years) and range of 26 to 416 weeks (0.5 to eight years). The mean maximum tumour size at resection was 8.3 cm, with a mean volume of 614 cm3 (range 9–2,113 cm3). Pain, typically dull and worse with sitting, was the most common presenting symptom in 85% of patients. The classic symptoms of cauda equina (saddle anaesthesia, bladder or bowel dysfunction) occurred in 70% patients (23 patients). Sacral chordoma should be considered in cases of back pain with coccydynia, especially with neurological symptoms. PMID:17205349

  13. Spinal extradural arteriovenous fistula with intradural drainage: three surgical cases with long-term follow-up.

    Science.gov (United States)

    Vázquez, Enrique J; Gefaell, Joaquín; Fernandez, Leticia; López, Eva; Areitio, Eduardo; Elexpuru, Jose A

    2017-06-01

    Spinal extradural arteriovenous fistulas (SEDAVF) with intradural drainage are uncommon vascular lesions that cause venous congestive myelopathy. We present three SEDAVF with intradural drainage treated with surgical disconnection of the intradural drainage via a single level laminectomy, followed by transarterial embolization (TAE) with Onyx if spontaneous thrombosis of the extradural fistula did not occur spontaneously. All patients improved their neurological condition and no congestive myelopathy recurrence was noted after mean follow-up of 84 months. We believe that SEDAVF with intradural drainage constitute a specific entity that should be included in the classifications of spinal arteriovenous malformations.

  14. Chordoma: clinical characteristics, management and prognosis of a case series of 25 patients

    OpenAIRE

    Ferraresi, Virginia; Nuzzo, Carmen; Zoccali, Carmine; Marandino, Ferdinando; Vidiri, Antonello; Salducca, Nicola; Zeuli, Massimo; Giannarelli, Diana; Cognetti, Francesco; Biagini, Roberto

    2010-01-01

    Abstract Background Adequate surgery still remains the only curative treatment of chordoma. Interesting clinical data on advanced disease with molecularly targeted therapies were reported. Methods We described the clinical outcome of a series of chordoma patients followed at Regina Elena National Cancer Centre of Rome from 2004 to 2008. Results Twenty-five consecutive patients with sacral (11 patients), spine (13 patients), and skull base (1 patient) chordoma went to our observation. Six pati...

  15. Efficacy of epidermal growth factor receptor targeting in advanced chordoma: case report and literature review.

    Science.gov (United States)

    Launay, Simon G; Chetaille, Bruno; Medina, Fanny; Perrot, Delphine; Nazarian, Serge; Guiramand, Jérôme; Moureau-Zabotto, Laurence; Bertucci, François

    2011-10-04

    Chordomas are very rare low-grade malignant bone tumors that arise from the embryonic rests of the notochord. They are characterized by slow growth and long history with frequent local relapses, and sometimes metastases. While chemotherapy is not efficient, imatinib has shown antitumor activity. We report on a 76-year-old patient with EGFR-overexpressing advanced chordoma that progressed on imatinib and subsequently responded to erlotinib during 12 months. We report the fourth case of advanced chordoma treated with an EGFR inhibitor. We also review the literature concerning the rationale and potential of EGFR targeting in chordoma.

  16. Efficacy of epidermal growth factor receptor targeting in advanced chordoma: case report and literature review

    Directory of Open Access Journals (Sweden)

    Guiramand Jérôme

    2011-10-01

    Full Text Available Abstract Background Chordomas are very rare low-grade malignant bone tumors that arise from the embryonic rests of the notochord. They are characterized by slow growth and long history with frequent local relapses, and sometimes metastases. While chemotherapy is not efficient, imatinib has shown antitumor activity. Case presentation We report on a 76-year-old patient with EGFR-overexpressing advanced chordoma that progressed on imatinib and subsequently responded to erlotinib during 12 months. Conclusions We report the fourth case of advanced chordoma treated with an EGFR inhibitor. We also review the literature concerning the rationale and potential of EGFR targeting in chordoma.

  17. ANTERIOR CERVICAL INTRADURAL ARACHNOID CYST - A RARE CAUSE OF SPINAL CORD COMPRESSION

    Directory of Open Access Journals (Sweden)

    Kollam Chandra Sekhar

    2016-07-01

    Full Text Available BACKGROUND Arachnoid cysts of spinal cord are relatively uncommon lesions. Most of them arise dorsal to the cord, and anteriorly placed intradural arachnoid cyst is a rare cause of cervical cord compression. To the best of our knowledge, only 30 cases were reported in the literature. We present a case of anterior cervical intradural arachnoid cyst with review of literature. METHODS We performed a literature search for anteriorly placed intradural arachnoid cysts in the cervical spinal cord through http://pubmed.com, a well-known worldwide internet medical address. To the best of our knowledge, only 30 cases were reported in the literature. We reviewed the literature with illustration of our case. We present a case of a 40-year-old male patient who presented with insidious onset of radicular pain. MRI cervical spine demonstrated cervical intradural cystic lesion extending from C2 to upper border of C4, lying anteriorly with compression over the cord. Cervical laminectomy followed by wide cyst fenestration and subtotal excision of cyst was done. Histopathological diagnosis was arachnoid cyst. RESULTS Patient totally recovered from his pain and sensory symptoms within a week and motor symptoms improved gradually over a period of six to eight weeks. With two years followup, patient had no further complaints. CONCLUSION Anterior cervical intradural arachnoid cysts are rare. These are amenable to resection through posterior approach safely with good postoperative recovery.

  18. The role of epidermal growth factor receptor in chordoma pathogenesis: a potential therapeutic target.

    Science.gov (United States)

    Shalaby, Asem; Presneau, Nadège; Ye, Hongtao; Halai, Dina; Berisha, Fitim; Idowu, Bernadine; Leithner, Andreas; Liegl, Bernadette; Briggs, Timothy R W; Bacsi, Krisztian; Kindblom, Lars-Gunnar; Athanasou, Nicholas; Amary, Maria Fernanda; Hogendoorn, Pancras C W; Tirabosco, Roberto; Flanagan, Adrienne M

    2011-02-01

    Chordoma, the molecular hallmark of which is T (brachyury), is a rare malignant bone tumour with a high risk of local recurrence and a tumour from which metastatic disease is a common late event. Currently, there is no effective drug therapy for treating chordomas, although there is evidence that some patients respond to the empirical use of epidermal growth factor receptor (EGFR) antagonists. The aim of this study was to determine the role of EGFR in the pathogenesis of chordoma. Paraffin-embedded material from 173 chordomas from 160 patients [sacro-coccygeal (n = 94), skull-based (n = 50), and mobile spine (n = 16)] was analysed by immunohistochemistry and revealed total EGFR expression in 69% of cases analysed. Of 147 informative chordomas analysed by FISH, 38% revealed high-level EGFR polysomy, 4% high-level polysomy with focal amplification, 18% low-level polysomy, and 39% disomy. Phospho-receptor tyrosine kinase array membranes showed EGFR activation in the chordoma cell line U-CH1 and all of the three chordomas analysed. Direct sequencing of EGFR (exons 18-21), KRAS, NRAS, HRAS (exons 2, 3), and BRAF (exons 11, 15) using DNA from 62 chordomas failed to reveal mutations. PTEN expression was absent by immunohistochemistry in 19 of 147 (13%) analysed chordomas, only one of which revealed high-level polysomy of EGFR. The EGFR inhibitor tyrphostin (AG 1478) markedly inhibited proliferation of the chordoma cell line U-CH1 in vitro and diminished EGFR phosphorylation in a dose-dependant manner, a finding supported by inhibition of phosphorylated Erk1/2. p-Akt was suppressed to a much lesser degree in these experiments. There was no reduction of T as assessed by western blotting. These data implicate aberrant EGFR signalling in the pathogenesis of chordoma. This study provides a strategy for patient stratification for treatment with EGFR antagonists. Copyright © 2010 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

  19. Endoscopic Transoral Resection of an Axial Chordoma: A Case Report

    Directory of Open Access Journals (Sweden)

    Taran S

    2015-11-01

    Full Text Available Upper cervical chordoma (UCC is rare condition and poses unique challenges to surgeons. Even though transoral approach is commonly employed, a minimally invasive technique has not been established. We report a 44-year old Malay lady who presented with a 1 month history of insidious onset of progressive neck pain without neurological symptoms. She was diagnosed to have an axial (C2 chordoma. Intralesional resection of the tumour was performed transorally using the Destandau endoscopic system (Storz, Germany. Satisfactory intralesional excision of the tumour was achieved. She had a posterior fixation of C1-C4 prior to that. Her symptoms improved postoperatively and there were no complications noted. She underwent adjuvant radiotherapy to minimize local recurrence. Endoscopic excision of UCC via the transoral approach is a safe option as it provides an excellent magnified view and ease of resection while minimizing the operative morbidity.

  20. Proton radiation therapy for clivus chordoma; Case report

    Energy Technology Data Exchange (ETDEWEB)

    Yoshii, Yoshihiko; Tsunoda, Takashi; Hyodo, Akio; Nose, Tadao (Tsukuba Univ., Ibaraki (Japan). Inst. of Clinical Medicine); Tsujii, Hirohiko; Tsuji, Hiroshi; Inada, Tetsuo; Maruhashi, Akira; Hayakawa, Yoshinori

    1993-03-01

    A 57-year-old male with clival chordoma developed severe hoarseness, dysphagia, and dysphonia 1 month after a second removal of the tumor. Magnetic resonance imaging demonstrated a mass 10 cm in diameter in the region of the middle clivus enhanced inhomogeneously by gadolinium-diethylenetriaminepenta-acetic acid, and a defect in the skull base. There was evidence of compression of the anterior surface of the pons. He received proton irradiation employing a pair of parallel opposed lateral proton beams. The dose aimed at the tumor mass was 75.5 Gy, to the pharyngeal wall less than 38 Gy, and to the anterior portion of the pons less than 30 Gy. Time dose and fractionation factor was calculated at 148. Thirty-one months following treatment, he was free of clinical neurological sequelae. Proton therapy should be considered in treatment planning following initial surgical removal or for inoperable clivus chordoma. (author).

  1. Computer Navigation-aided Resection of Sacral Chordomas

    Directory of Open Access Journals (Sweden)

    Yong-Kun Yang

    2016-01-01

    Full Text Available Background: Resection of sacral chordomas is challenging. The anatomy is complex, and there are often no bony landmarks to guide the resection. Achieving adequate surgical margins is, therefore, difficult, and the recurrence rate is high. Use of computer navigation may allow optimal preoperative planning and improve precision in tumor resection. The purpose of this study was to evaluate the safety and feasibility of computer navigation-aided resection of sacral chordomas. Methods: Between 2007 and 2013, a total of 26 patients with sacral chordoma underwent computer navigation-aided surgery were included and followed for a minimum of 18 months. There were 21 primary cases and 5 recurrent cases, with a mean age of 55.8 years old (range: 35-84 years old. Tumors were located above the level of the S3 neural foramen in 23 patients and below the level of the S3 neural foramen in 3 patients. Three-dimensional images were reconstructed with a computed tomography-based navigation system combined with the magnetic resonance images using the navigation software. Tumors were resected via a posterior approach assisted by the computer navigation. Mean follow-up was 38.6 months (range: 18-84 months. Results: Mean operative time was 307 min. Mean intraoperative blood loss was 3065 ml. For computer navigation, the mean registration deviation during surgery was 1.7 mm. There were 18 wide resections, 4 marginal resections, and 4 intralesional resections. All patients were alive at the final follow-up, with 2 (7.7% exhibiting tumor recurrence. The other 24 patients were tumor-free. The mean Musculoskeletal Tumor Society Score was 27.3 (range: 19-30. Conclusions: Computer-assisted navigation can be safely applied to the resection of the sacral chordomas, allowing execution of preoperative plans, and achieving good oncological outcomes. Nevertheless, this needs to be accomplished by surgeons with adequate experience and skill.

  2. On a Rare Cutaneous Metastasis from a Sacrococcygeal Chordoma

    Directory of Open Access Journals (Sweden)

    Alessandro D’Amuri

    2017-01-01

    Full Text Available Chordomas are rare malignant tumors of notochordal origin and are rare locally aggressive ones with a metastatic potential. The skin rarely is seen as metastatic site. We describe a case of an adult woman with cutaneous metastasis of a primary sacral chordoma excised ten years before, which appeared as a painless cutaneous mass located in the dorsal region. Once removed, the surgical specimen was formalin fixed and in paraffin embedded. Sections were stained with haematoxylin-eosin, and histochemical and immunohistochemical investigations were performed. Histologically, the neoplasia was characterized by cords or single tumor cells with an abundant myxoid stroma, conspicuous pale vacuolated cytoplasm (the classic “physaliphorous cells”, and mild nuclear atypia. Mitotic activity was scanty. At immunohistochemistry, the tumor cells were diffusely positive for S-100 protein, pan-keratins, EMA, and vimentin. A diagnosis of cutaneous metastasis of chordoma was performed. This case illustrates a diagnostic challenge because of the unusual presentation of an already rare tumor.

  3. Phase II study of imatinib in advanced chordoma.

    Science.gov (United States)

    Stacchiotti, Silvia; Longhi, Alessandra; Ferraresi, Virginia; Grignani, Giovanni; Comandone, Alessandro; Stupp, Roger; Bertuzzi, Alexia; Tamborini, Elena; Pilotti, Silvana; Messina, Antonella; Spreafico, Carlo; Gronchi, Alessandro; Amore, Paola; Vinaccia, Vincenza; Casali, Paolo Giovanni

    2012-03-20

    To explore the antitumor activity of imatinib in patients with advanced platelet-derived growth factor β (PDGFB)/PDGF receptor β (PDGFRB)-positive chordomas. In a collaborative Italian-Swiss, prospective, phase II clinical study conducted from November 2004 through April 2006, 56 patients with advanced PDGFB and/or PDGFRB chordoma received 800 mg/d of imatinib until progression. The primary end point was the overall tumor response rate (ORR), defined by RECIST. Secondary, exploratory end points included tissue response (ie, changes in tumor density or signal intensity/contrast enhancement, and/or [18F]-fluorodeoxyglucose positron emission tomography [PET] uptake), overall survival, progression-free survival (PFS), and pain score. Among 50 patients evaluable by RECIST, the best response was one partial response (PR) obtained at 6 months (ORR, 2%). There were 35 patients with stable disease (SD, 70%) and a 64% clinical benefit rate (ie, RECIST complete response + PR + SD ≥ 6 months). A minor dimensional response (chordoma to date. It confirms anecdotal evidence that imatinib has antitumor activity in this orphan disease, and therefore, it is worth further investigation.

  4. Two-Stage en bloc Resection of Multilevel Cervical Chordomas With Vertebral Artery Preservation: Operative Technique.

    Science.gov (United States)

    Wang, Xingwen; Eichbaum, Eldan; Jian, Fengzeng; Chou, Dean

    2017-08-09

    En bloc excision of cervical chordoma is a technically complex procedure, due to the involvement and closeness of the tumor to the spinal cord, cervical nerve roots, and vertebral arteries. Studies have previously shown that en bloc excision of chordomas with negative margins improves local control and prolongs disease-free survival compared with intralesional excision. True en bloc spondylectomy in the cervical spine is not feasible since bilateral vertebral artery sacrifice is not possible. However, for lateralized tumors, en bloc excision of chordoma can be performed with unilateral vertebral artery preservation by parasagittal osteotomy. To describe the operative technique of performing en bloc excision of cervical chordoma via parasagittal osteotomy. Four patients underwent en bloc excision of multilevel cervical chordomas via parasagittal osteotomy between 2008 and 2016. These 4 cases of chordoma were at the upper-cervical, mid-cervical, and cervicothoracic regions. We analyzed the tumor location, oncological staging, surgical technique, and perioperative complications. All 4 patients underwent en bloc excision of chordoma with expandable cage reconstruction and posterior instrumentation. Cervical nerve roots were sacrificed in 2 patients, and vertebral artery ligation was performed in 3 patients. Complications include new neurologic deficit, implant failure, and pharyngeal erosion after radiation. No tumor recurrence was seen. Parasagittal osteotomy is a useful alternative to en bloc spondylectomy in the treatment of lateralized multilevel cervical chordoma, preserving one vertebral artery while still achieving an en bloc resection. Copyright © 2017 by the Congress of Neurological Surgeons

  5. Treatment of Recurrent Chordomas by Percutaneous Ethanol Injection Therapy and Radiation Therapy

    International Nuclear Information System (INIS)

    Nakajo, M.; Ohkubo, K.; Fukukura, Y.; Nandate, T.; Nakajo, M.

    2006-01-01

    We report a case of recurrent sacral chordomas that have been successfully controlled by the combination therapy of percutaneous ethanol injection therapy (PEIT) and radiation therapy in a 71-year-old man. PEIT may be one of the adjuvant therapies for recurrent chordomas

  6. Conus medullaris syndrome due to an intradural disc herniation: A case report

    Directory of Open Access Journals (Sweden)

    Chaudhary Kshitij

    2008-01-01

    Full Text Available A 70-year-old male patient developed acute paraplegia due to conus medullaris compression secondary to extrusion of D12-L1 disc. After negative epidural examination intraoperatively, a durotomy was performed and an intradural disc fragment was excised. Patient did not regain ambulatory status at two-year follow-up. Intraoperative finding of negative extradural compression, tense swollen dura and CSF leak from ventral dura should alert the surgeon for the possibility of intradural disc herniation. A routine preoperative MRI is misleading and a high index of suspicion helps to avoid a missed diagnosis.

  7. Clival chordoma: a single-centre outcome analysis.

    Science.gov (United States)

    Jägersberg, Max; El Rahal, Amir; Dammann, Philipp; Merkler, Doron; Weber, Damien Charles; Schaller, Karl

    2017-10-01

    The treatment of clival chordomas remains challenging. Total tumour resection is often impossible without hampering adjacent anatomical structures and causing functional sequelae. On the other hand, chordomas show limited response to non-surgical treatment modalities. Up to now, no well-established interdisciplinary treatment algorithms for clival chordomas exist. In this regard, we analysed the data from all patients that underwent interdisciplinary treatment for clival chordoma in our institution over the last 10 years. Retrospective report of all patients treated at the authors' institution from 2005 to 2015. Thirteen patients underwent 24 surgeries, of which 2 (8%) were gross total resections and 22 (92%) incomplete resections. Neurological deterioration, endocrinological disturbances and other surgical complications were observed in six (25%), three (13%) and nine (38%) cases, respectively. Three surgeries (13%) led to an improvement of the initial preoperative neurological condition. All patients were discussed on the interdisciplinary tumour board and all underwent one type of radiotherapy following initial surgery: proton beam in 11 cases (85%) and photon beam in two (15%) cases. In the course of their recurrent disease, three patients (23%) received systemic therapy (imatinib, pazopanib and nivolumab). One patient received a personalised cellular immunotherapy. One patient (8%) was lost to follow-up. Of the remaining 12 patients, four patients (33%) died in the period of analysis; all deaths were chordoma-related. The 5-year cumulative survival rate was 83% (52-97%, CI 95%), 5-year progression-free survival rate was 53% (26-79%, CI 95%). The eight patients (66%) still alive had favourable outcome (KPS, 90 ± 10.7%). SF36 analysis among the survivors revealed 43 points for the Physical Component Summary (12% above, 38% at and 50% below the general population norm) and 47 points for the Mental Component Summary (25% above, 38% at and 38% below). Our

  8. Spontaneous Cervical Intradural Disc Herniation Associated with Ossification of Posterior Longitudinal Ligament

    Directory of Open Access Journals (Sweden)

    Dachuan Wang

    2014-01-01

    Full Text Available Intradural herniation of a cervical disc is rare; less than 35 cases have been reported to date. A 52-year-old man with preexisting ossification of posterior longitudinal ligament developed severe neck pain with Lt hemiparesis while asleep. Neurological exam was consistent with Brown-Séquard syndrome. Magnetic resonance images showed a C5-6 herniated disc that was adjacent to the ossified ligament and indenting the cord. The mass was surrounded by cerebrospinal fluid signal intensity margin, and caudally the ventral dura line appears divided into two, consistent with the “Y-sign” described by Sasaji et al. Cord edema were noted. Because of preexisting canal stenosis and spinal cord at risk, a laminoplasty was performed, followed by an anterior C6 corpectomy. Spot-weld type adhesions of the posterior longitudinal ligament to the dura was noted, along with a longitudinal tear in the dura. An intradural extra-arachnoid fragment of herniated disc was removed. Clinical exam at 6 months after surgery revealed normal muscle strength but persistent mild paresthesias. It is difficult to make a definite diagnosis of intradural herniation preoperatively; however, the clinical findings and radiographic signs mentioned above are suggestive and should alert the surgeon to look for an intradural fragment.

  9. Immunophenotypic features of dedifferentiated skull base chordoma: An insight into the intratumoural heterogeneity

    Directory of Open Access Journals (Sweden)

    Kelvin Manuel Pińa Batista

    2017-12-01

    Full Text Available Chordomas are rare and low-grade malignant solid tumours, despite their histologically benign appearance, that arise in the bone from embryonic notochordal vestiges of the axial skeleton, a mesoderm-derived structure that is involved in the process of neurulation and embryonic development. Chordomas occurring in the skull base tend to arise in the basiocciput along the clivus. Three major morphological variants have been described (classical, chondroid, and atypical/dedifferentiated. The pathogenesis and molecular mechanisms involved in chordoma development remain uncertain. From a pathological standpoint, the microenvironment of a chordoma is heterogeneous, showing a dual epithelial-mesenchymal differentiation. These tumours are characterised by slow modality of biologic growth, local recurrence, low incidence of metastasis rates, and cancer stem cell (CSC phenotype. The main molecular findings are connected with brachyury immunoexpression and activation of the downstream Akt and mTOR signalling pathways. The differentiation between typical and atypical chordomas is relevant because the tumoural microenvironment and prognosis are partially different. This review provides an insight into the recent and relevant concepts and histochemical markers expressed in chordomas, with special emphasis on dedifferentiated chordomas and their prognostic implications.

  10. Imatinib in advanced chordoma: A retrospective case series analysis.

    Science.gov (United States)

    Hindi, Nadia; Casali, Paolo G; Morosi, Carlo; Messina, Antonella; Palassini, Elena; Pilotti, Silvana; Tamborini, Elena; Radaelli, Stefano; Gronchi, Alessandro; Stacchiotti, Silvia

    2015-11-01

    Imatinib showed activity in 50 chordoma patients treated within a Phase II study. In that study, 70% of patients remained with stable disease (SD), median progression free survival (PFS) was 9 months and median overall survival (OS) was 34 months. We now report on a retrospective series of PDGFB/PDGFRB positive advanced chordoma patients treated with imatinib as a single agent within a compassionate-use programme at Istituto Nazionale Tumori, Milan, Italy (INT) between August 2002 and November 2010, when the programme was closed. 48 patients were consecutively treated with imatinib 800 mg/d. All patients had inoperable and progressive disease before starting imatinib. Demographics, treatment duration, toxicity and response rate by Response Evaluation Criteria in Solid Tumors (RECIST) were retrospectively recorded. The median duration of therapy was 7 months (1-46.5). No patient is on therapy at present. 46 patients were evaluable for response. No partial responses were detected. Best response was: stable disease 34 (74%), progressive disease 12 (26%). At a median follow-up of 24.5 months (0.5-117), median PFS was 9.9 months (95% confidence interval (CI) 6.7-13). Eight patients (16.5%) remained on therapy >18 months and 10 patients (21%) remained progression-free >18 months. Median OS was 30 months (95% CI 20-40), with 24 (50%) patients dead at the time of the present analysis. We confirm the activity of imatinib in locally advanced and metastatic chordoma, in terms of >70% tumour growth arrest in previously progressive patients. Median duration of response lasted almost 10 months, with >20% of patients progression-free at 18+ months. Copyright © 2015 Elsevier Ltd. All rights reserved.

  11. Chordoma: 6 years' experience at a tertiary centre

    International Nuclear Information System (INIS)

    Agrawal, P.P.; Bahadur, A.K.; Mohanta, P.K.; Singh, K.; Rathi, A.K.

    2006-01-01

    Nine patients with a histologically proven diagnosis of chordoma seen at the Department of Radiation Oncology, Maulana Azad Medical College and Lok Nayak Hospital between January 1999 and December 2004 were retrospectively reviewed with respect to age, sex, presentation, location of tumour, treatment, response, recurrence, metastasis and follow up. Chordoma constituted 0.07% of total cancer cases registered over 6 years. Out of nine patients, eight were males and one was female with median age at time of diagnosis 52 years (range 34-68 years). All had sacrococcygeal lesions except one who had a spheno-occipital lesion. Seven patients had undergone either subtotal or gross total resection whereas only biopsy had been carried out in two of them. All patients received radiation therapy, seven in a postoperative setting and two for palliation. Follow-up period ranged from 2 to 50 months. Four patients died - the first after fourth fraction of radiation, second after 10 days of treatment, third of progressive lesion in sphenoidal region despite resection and radiation and fourth of local recurrence in the sacrococcyx. One patient developed distant metastases in the lungs and subcutaneous tissue over the scalp along with local recurrence; he is still alive. Two patients are locally free of disease whereas the other two were lost to follow up. The present analysis was undertaken to review our institutional experience with an aim to provide a practical approach to these tumours. In this report, these cases are discussed and the published works have been reviewed for the optimal management of patients with chordoma Copyright (2006) Blackwell Publishing Asia Pty Ltd

  12. Intraoperative Spinal Navigation for the Removal of Intradural Tumors: Technical Notes.

    Science.gov (United States)

    Stefini, Roberto; Peron, Stefano; Mandelli, Jaime; Bianchini, Elena; Roccucci, Paolo

    2017-08-05

    In recent years, spinal surgery has incorporated the many advantages of navigation techniques to facilitate the placement of pedicle screws during osteosynthesis, mainly for degenerative diseases. However, spinal intradural tumors are not clearly visible by intraoperative fluoroscopy or computed tomography scans, thereby making navigation necessary. To evaluate the use of spinal navigation for the removal of intradural and spinal cord tumors using spinal magnetic resonance imaging (MRI) merged with intraoperative 3-dimensional (3-D) fluoro images. After fixing the patient reference frame on the spinous process, the 3-D fluoro images were obtained in the surgical room. Using this image as the reference, the preoperative volumetric MRI images and intraoperative 3-D fluoro images were merged using automated software or manually. From January to July 2016, we performed 10 navigated procedures for intradural spinal tumors by merging MRI and 3-D fluoro images. Nine patients had an intradural extramedullary tumor, 6 had neurinomas, and 3 had meningiomas; 1 patient had an intramedullary spinal cord metastasis. The surgically demonstrated benefits of spinal navigation for the removal of intradural tumors include the decreased risk of surgery at the wrong spinal level, a minimal length of skin incision and muscle strip, and a reduction in bone removal extension. Furthermore, this technique offers the advantage of opening the dura as much as is necessary and, in the case of intrinsic spinal cord tumors, it allows the tumor to be centered. Otherwise, this would not be visible, thus enabling the precise level and the posterior midline sulcus to be determined when performing a mielotomy. Copyright © 2017 by the Congress of Neurological Surgeons

  13. Cervical chordoma with vertebral artery encasement mimicking neurofibroma: MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Mortele, B.; Lemmerling, M.; Mortele, K.; Verstraete, K.; Defreyne, L.; Kunnen, M. [Department of Radiology, University Hospital, Gent (Belgium); Vandekerckhove, T. [Department of Neurosurgery, University Hospital, Gent (Belgium)

    2000-06-01

    A case of cervical chordoma in a 36-year-old white man with hypoesthesia in the neck and right shoulder, neck pain, and restricted neck mobility is presented. Plain radiographs of the cervical spine showed radiolucency of the body of C2 on the right side and enlargement of the right intervertebral foramen at C2-C3 level. Tumor encasement of the vertebral artery was demonstrated by MR imaging and confirmed by conventional arteriography. This proved to be particularly important for preoperative assessment. (orig.)

  14. Cervical chordoma with vertebral artery encasement mimicking neurofibroma: MRI findings

    International Nuclear Information System (INIS)

    Mortele, B.; Lemmerling, M.; Mortele, K.; Verstraete, K.; Defreyne, L.; Kunnen, M.; Vandekerckhove, T.

    2000-01-01

    A case of cervical chordoma in a 36-year-old white man with hypoesthesia in the neck and right shoulder, neck pain, and restricted neck mobility is presented. Plain radiographs of the cervical spine showed radiolucency of the body of C2 on the right side and enlargement of the right intervertebral foramen at C2-C3 level. Tumor encasement of the vertebral artery was demonstrated by MR imaging and confirmed by conventional arteriography. This proved to be particularly important for preoperative assessment. (orig.)

  15. Response to erlotinib in a patient with treatment refractory chordoma.

    Science.gov (United States)

    Singhal, Nimit; Kotasek, Dusan; Parnis, Francis X

    2009-11-01

    Chordomas are rare tumors arising from the axial skeleton. The disease is characterized by slow local growth, frequent local recurrences, and rare systemic spread. Surgery and local radiation remains the mainstay of treatment with minimal role of systemic therapy. Imatinib has been shown to be active in a phase II trial with symptomatic and radiological responses. We report a case where treatment with erlotinib, an epidermal growth factor receptor tyrosine kinase inhibitor, induced symptomatic and radiological response in a patient with disease refractory to imatinib and vascular disrupting agent.

  16. Cervical chondroid chordoma in a standard dachshund: a case report

    Directory of Open Access Journals (Sweden)

    Stigen Øyvind

    2011-10-01

    Full Text Available Abstract A ten-year-old male standard dachshund was presented with a history of neck pain and progressive gait disturbances. Following a neurological examination and diagnostic imaging, including CT, a neoplastic lesion involving the third and fourth cervical vertebrae was suspected. The lesion included an extradural mass on the right side of the spinal canal causing a local compression of the cervical cord. Surgery, using a modified dorsal laminectomy procedure, was performed in order to decompress the cervical spinal cord. Histopathological examination of the extradural mass indicated that the tumour was a chondroid chordoma. Following discharge, the quality of life for the dog was very good for a sustained period, but clinical signs recurred at 22 months. The dog was euthanased 25 months post-surgery. On post-mortem examination, a regrowth of neoplastic tissue was found to have infiltrated the bone and spinal cord at C3-C4. This is the first report to show that palliative surgery can offer successful long-lasting treatment of chondroid chordoma of the cervical spine in the dog.

  17. Stereotactic radiosurgery for intradural spine tumors using cone-beam CT image guidance.

    Science.gov (United States)

    Monserrate, Andrés; Zussman, Benjamin; Ozpinar, Alp; Niranjan, Ajay; Flickinger, John C; Gerszten, Peter C

    2017-01-01

    OBJECTIVE Cone-beam CT (CBCT) image guidance technology has been widely adopted for spine radiosurgery delivery. There is relatively little experience with spine radiosurgery for intradural tumors using CBCT image guidance. This study prospectively evaluated a series of intradural spine tumors treated with radiosurgery. Patient setup accuracy for spine radiosurgery delivery using CBCT image guidance for intradural spine tumors was determined. METHODS Eighty-two patients with intradural tumors were treated and prospectively evaluated. The positioning deviations of the spine radiosurgery treatments in patients were recorded. Radiosurgery was delivered using a linear accelerator with a beam modulator and CBCT image guidance combined with a robotic couch that allows positioning correction in 3 translational and 3 rotational directions. To measure patient movement, 3 quality assurance CBCTs were performed and recorded in 30 patients: before, halfway, and after the radiosurgery treatment. The positioning data and fused images of planning CT and CBCT from the treatments were analyzed to determine intrafraction patient movements. From each of 3 CBCTs, 3 translational and 3 rotational coordinates were obtained. RESULTS The radiosurgery procedure was successfully completed for all patients. Lesion locations included cervical (22), thoracic (17), lumbar (38), and sacral (5). Tumor histologies included schwannoma (27), neurofibromas (18), meningioma (16), hemangioblastoma (8), and ependymoma (5). The mean prescription dose was 17 Gy (range 12-27 Gy) delivered in 1-3 fractions. At the halfway point of the radiation, the translational variations and standard deviations were 0.4 ± 0.5, 0.5 ± 0.8, and 0.4 ± 0.5 mm in the lateral (x), longitudinal (y), and anteroposterior (z) directions, respectively. Similarly, the variations immediately after treatment were 0.5 ± 0.4, 0.5 ± 0.6, and 0.6 ± 0.5 mm along x, y, and z directions, respectively. The mean rotational angles were 0

  18. Chordoma of the petrous apex - a case report and review of the literature

    International Nuclear Information System (INIS)

    Loureiro, Ricardo; Leal Junior, Osvaldo S.; Loureiro, Lautonio Junior; Buril, Marlus V.M.

    1998-01-01

    Chordomas are rare tumours arising from remnants of the embryologic notochord, typically at a midline position. Although 35-40% of these lesions are intracranial, these tumors answer for less than 1% of all intracranial tumors. The intracranial chordomas originate most frequently from the clival region at the midline. Nevertheless eventually may arise off the midline primarily in petrous apex or, very rarely, in paranasal sinuses. The authors report a case of histopathologically proved intracranial chordoma that arose atypical site in the petrous apex. The computed tomographic and magnetic resonance imaging finding were similar to those observed in midline chordomas. The computed tomographic examination revealed a well-defined soft tissue mass associated with bone destruction and foci of calcification. The magnetic resonance imaging study demonstrated a growing extra-axial formation that appeared with hypo-intensity of signal on T1-weighted images, hyperintensity on T2-weighted images and heterogeneous enhancement after paramagnetic agent injection. (author)

  19. Upper cervical spine chordoma of C2–C3

    OpenAIRE

    Jiang, Liang; Liu, Zhong Jun; Liu, Xiao Guang; Ma, Qing Jun; Wei, Feng; Lv, Yang; Dang, Geng Ting

    2009-01-01

    Chordoma is a rare low-grade malignant neoplasm derived from the remnants of the embryonic notochord. This locally invasive neoplasm is subject to recurrence after treatment. The median survival time is estimated to be 6.3 years. Various treatment approaches have been attempted, including radical excision, radiotherapy and chemotherapy. Treatment outcome is significantly influenced by the size and site of the chordoma. Recently, Imatinib, a molecular-targeted agent, has been shown to have ant...

  20. Metastatic Chordoma: Report of the Two Cases and Review of the Literature.

    Science.gov (United States)

    Rohatgi, Saurabh; Ramaiya, Nikhil H; Jagannathan, Jyothi P; Howard, Stephanie A; Shinagare, Atul B; Krajewski, Katherine M

    2015-06-01

    Chordomas are rare malignant bone tumours with a predilection for the axial skeleton, especially the sacrum and skull base. Median survival in patients with metastatic disease is usually dismal. Treatment is challenging due to the propensity for local recurrence, metastatic disease as well as lack of clear consensus regarding the optimal management. Our case report highlights two cases of sacral chordoma with locally recurrent and widespread metastatic disease, stable on molecular targeted therapy.

  1. Efficacy of epidermal growth factor receptor targeting in advanced chordoma: case report and literature review

    OpenAIRE

    Launay, Simon G; Chetaille, Bruno; Medina, Fanny; Perrot, Delphine; Nazarian, Serge; Guiramand, J?r?me; Moureau-Zabotto, Laurence; Bertucci, Fran?ois

    2011-01-01

    Abstract Background Chordomas are very rare low-grade malignant bone tumors that arise from the embryonic rests of the notochord. They are characterized by slow growth and long history with frequent local relapses, and sometimes metastases. While chemotherapy is not efficient, imatinib has shown antitumor activity. Case presentation We report on a 76-year-old patient with EGFR-overexpressing advanced chordoma that progressed on imatinib and subsequently responded to erlotinib during 12 months...

  2. Auto-immune thyroid dysfunction induced by tyrosine kinase inhibitors in a patient with recurrent chordoma

    OpenAIRE

    Eroukhmanoff, Juliette; Castinetti, Frederic; Penel, Nicolas; Salas, Sebastien

    2016-01-01

    Background While hypothyroidism has frequently been reported with the use of TKIs, the thyroid-stimulating hormone (TSH) suppressing effect of TKIs is rare, except for thyroiditis. We describe a case with progressive recurrent chordoma who initially became hyperthyroid in a context of autoimmunity under sorafenib treatment and later under imatinib treatment. Case presentation A 57-year-old man with lumbar chordoma began daily treatment of 800?mg sorafenib. He did not have any other medication...

  3. Efficacy of pazopanib and sunitinib in advanced axial chordoma: a single reference centre case series

    OpenAIRE

    Lipplaa, Astrid; Dijkstra, Sander; Gelderblom, Hans

    2016-01-01

    Background Chordomas are rare malignant tumours of the axial skeleton and skull base supposed to arise from cellular remnants of the notochord. These tumours have the potential to metastasize (30?40?%), usually in the later course of the disease. However, the greatest morbidity is usually a result of loco-regional recurrence with infiltration and destruction of surrounding bone and soft tissue. Patients with unresectable or metastatic chordoma are faced with a poor prognosis since cytotoxic c...

  4. Immunohistochemical analysis of receptor tyrosine kinase signal transduction activity in chordoma.

    Science.gov (United States)

    Fasig, J H; Dupont, W D; LaFleur, B J; Olson, S J; Cates, J M M

    2008-02-01

    Currently, there are no effective chemotherapeutic protocols for chordoma. Reports of receptor tyrosine kinase (RTK) expression in chordoma suggest that these tumours may respond to kinase inhibitor therapy. However, RTK signalling activity has not been extensively investigated in chordoma. A tissue microarray containing 21 cases of chordoma was analysed for expression of a number of proteins involved in signal transduction from RTKs by immunohistochemistry. Platelet-derived growth factor receptor-beta, epidermal growth factor receptor (EGFR), KIT and HER2 were detected in 100%, 67%, 33% and 0% of cases, respectively. Platelet-derived growth factor receptor-beta staining was of moderate-to-strong intensity in 20 of 21 cases. In contrast, KIT immunoreactivity was weak and focal in each of the seven positive cases. Total EGFR staining was variable; weak staining for phosphorylated EGFR was detected in nine cases. Phosphorylated isoforms of p44/42 mitogen-activated protein kinase, Akt and STAT3, indicative of tyrosine kinase activity, were detected in 86%, 76% and 67% of cases, respectively. Chordomas commonly express RTKs and activated signal transduction molecules. Although there were no statistically significant correlations between the expression of any of the markers studied and disease-free survival or tumour location, the results nonetheless indicate that chordomas may respond to RTK inhibitors or modulators of other downstream signalling molecules.

  5. Proton Therapy for Reirradiation of Progressive or Recurrent Chordoma

    Energy Technology Data Exchange (ETDEWEB)

    McDonald, Mark W., E-mail: mmcdona2@iuhealth.org [Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana (United States); Indiana University Health Proton Therapy Center, Bloomington, Indiana (United States); Linton, Okechuckwu R. [Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana (United States); Shah, Mitesh V. [Department of Neurosurgery, Indiana University School of Medicine, Indianapolis, Indiana (United States)

    2013-12-01

    Purpose: To report the results in patients reirradiated with proton therapy for recurrent or progressive chordoma, with or without salvage surgery. Methods and Materials: A retrospective review of 16 consecutive patients treated from 2005 to 2012 was performed. All patients had received at least 1 prior course of radiation therapy to the same area, and all but 1 patient had at least 1 surgical resection for disease before receiving reirradiation. At the time of recurrence or progression, half of the patients underwent additional salvage surgery before receiving reirradiation. The median prior dose of radiation was 75.2 Gy (range, 40-79.2 Gy). Six patients had received prior proton therapy, and the remainder had received photon radiation. The median gross tumor volume at the time of reirradiation was 71 cm{sup 3} (range, 0-701 cm{sup 3}). Reirradiation occurred at a median interval of 37 months after prior radiation (range, 12-129 months), and the median dose of reirradiation was 75.6 Gy (relative biological effectiveness [RBE]) (range. 71.2-79.2 Gy [RBE]), given in standard daily fractionation (n=14) or hyperfractionation (n=2). Results: The median follow-up time was 23 months (range, 6-63 months); it was 26 months in patients alive at the last follow-up visit (range, 12-63 months). The 2-year estimate for local control was 85%, overall survival 80%, chordoma-specific survival 88%, and development of distant metastases 20%. Four patients have had local progression: 3 in-field and 1 marginal. Late toxicity included grade 3 bitemporal lobe radionecrosis in 1 patient that improved with hyperbaric oxygen, a grade 4 cerebrospinal fluid leak with meningitis in 1 patient, and a grade 4 ischemic brainstem stroke (out of radiation field) in 1 patient, with subsequent neurologic recovery. Conclusions: Full-dose proton reirradiation provided encouraging initial disease control and overall survival for patients with recurrent or progressive chordoma, although additional

  6. Skull base chordomas: analysis of dose-response characteristics

    International Nuclear Information System (INIS)

    Niemierko, Andrzej; Terahara, Atsuro; Goitein, Michael

    1997-01-01

    Objective: To extract dose-response characteristics from dose-volume histograms and corresponding actuarial survival statistics for 115 patients with skull base chordomas. Materials and Methods: We analyzed data for 115 patients with skull base chordoma treated with combined photon and proton conformal radiotherapy to doses in the range 66.6Gy - 79.2Gy. Data set for each patient included gender, histology, age, tumor volume, prescribed dose, overall treatment time, time to recurrence or time to last observation, target dose-volume histogram, and several dosimetric parameters (minimum/mean/median/maximum target dose, percent of the target volume receiving the prescribed dose, dose to 90% of the target volume, and the Equivalent Uniform Dose (EUD). Data were analyzed using the Kaplan-Meier survivor function estimate, the proportional hazards (Cox) model, and parametric modeling of the actuarial probability of recurrence. Parameters of dose-response characteristics were obtained using the maximum likelihood method. Results: Local failure developed in 42 (36%) of patients, with actuarial local control rates at 5 years of 59.2%. The proportional hazards model revealed significant dependence of gender on the probability of recurrence, with female patients having significantly poorer prognosis (hazard ratio of 2.3 with the p value of 0.008). The Wilcoxon and the log-rank tests of the corresponding Kaplan-Meier recurrence-free survival curves confirmed statistical significance of this effect. The Cox model with stratification by gender showed significance of tumor volume (p=0.01), the minimum target dose (p=0.02), and the EUD (p=0.02). Other parameters were not significant at the α level of significance of 0.05, including the prescribed dose (p=0.21). Parametric analysis using a combined model of tumor control probability (to account for non-uniformity of target dose distribution) and the Weibull failure time model (to account for censoring) allowed us to estimate

  7. Thoracolumbar intradural disc herniation in eight dogs: clinical, low-field magnetic resonance imaging, and computed tomographic myelography findings.

    Science.gov (United States)

    Tamura, Shinji; Doi, Shoko; Tamura, Yumiko; Takahashi, Kuniaki; Enomoto, Hirokazu; Ozawa, Tsuyoshi; Uchida, Kazuyuki

    2015-01-01

    Intradural disc herniation is a rarely reported cause of neurologic deficits in dogs and few published studies have described comparative imaging characteristics. The purpose of this retrospective cross sectional study was to describe clinical and imaging findings in a group of dogs with confirmed thoracolumbar intradural disc herniation. Included dogs were referred to one of four clinics, had acute mono/paraparesis or paraplegia, had low field magnetic resonance imaging (MRI) and/or computed tomographic myelography, and were diagnosed with thoracolumbar intradural disc herniation during surgery. Eight dogs met inclusion criteria. The prevalence of thoracolumbar intradural disc herniation amongst the total population of dogs that developed a thoracolumbar intervertebral disc herniation and that were treated with a surgical procedure was 0.5%. Five dogs were examined using low-field MRI. Lesions that were suspected to be intervertebral disc herniations were observed; however, there were no specific findings indicating that the nucleus pulposus had penetrated into the subarachnoid space or into the spinal cord parenchyma. Thus, the dogs were misdiagnosed as having a conventional intervertebral disc herniation. An intradural extramedullary disc herniation (three cases) or intramedullary disc herniation (two cases) was confirmed during surgery. By using computed tomographic myelography (CTM) for the remaining three dogs, an intradural extramedullary mass surrounded by an accumulation of contrast medium was observed and confirmed during surgery. Findings from this small sample of eight dogs indicated that CTM may be more sensitive for diagnosing canine thoracolumbar intradural disc herniation than low-field MRI. © 2014 American College of Veterinary Radiology.

  8. Osseous metastases of chordoma: imaging and clinical findings

    Energy Technology Data Exchange (ETDEWEB)

    Chang, Connie; Torriani, Martin; Bredella, Miriam [Massachusetts General Hospital, Division of Musculoskeletal Imaging and Intervention, Department of Radiology, Boston, MA (United States); Chebib, Ivan [Massachusetts General Hospital, Department of Pathology, Boston, MA (United States)

    2017-03-15

    To describe the imaging and clinical characteristics of chordoma osseous metastases (COM). Our study was IRB approved and HIPAA compliant. A retrospective search of our pathology database for pathology-proven COM yielded 15 patients who had undergone MRI, CT, bone scan, and/or FDG-PET/CT. The imaging and clinical features of the COMs were recorded. A control group of age and gender matched chordoma patients without osseous metastasis was evaluated. The COM mean maximal dimension was 6.4 ± 4.0 cm. The majority (60%) of patients had one lesion. Extra-osseous soft tissue component was present in 85% and was larger than intra-osseous component in 76%. On MRI the lesions were heterogeneous but predominantly T2 hyperintense with hypointense septae, and with variable enhancement. On CT the lesions were typically destructive or permeative; calcifications were rare. The extent of the soft tissue component was isodense to muscle on CT and therefore better evaluated on MRI. COM was in a body part contiguous to the site of the primary tumor. Compared to the controls, COM patients were more likely to have local recurrence (P = 0.0009) and positive resection margins (P = 0.002). At 1 year, 33% of COM patients were deceased and 13% had progressive metastases. COM are associated with large extra-osseous soft tissue components, which are better visualized by MRI. They are often located in a body part contiguous to the site of the primary tumor, portend poor prognosis, and are associated with positive resection margins and local recurrence. (orig.)

  9. Multidisciplinary management of clival chordomas; long-term clinical outcome in a single-institution consecutive series

    DEFF Research Database (Denmark)

    Förander, Petter; Bartek, Jiri; Fagerlund, Michael

    2017-01-01

    OBJECTIVE: Chordomas of the skull base have high recurrence rates even after radical resection and adjuvant radiotherapy. We evaluate the long-term clinical outcome using multidisciplinary management in the treatment of clival chordomas. METHODS: Between 1984 and 2015, 22 patients diagnosed with ...

  10. Molecular targeted therapies in advanced or metastatic chordoma patients: facts and hypotheses.

    Science.gov (United States)

    Lebellec, Loïc; Aubert, Sébastien; Zaïri, Fahed; Ryckewaert, Thomas; Chauffert, Bruno; Penel, Nicolas

    2015-07-01

    Chordomas, derived from undifferentiated notochordal remnants, represent less than 4% of bone primary tumors. Despite surgery followed by radiotherapy, local and metastatic relapses are frequent. In case of locally advanced or metastatic chordomas, medical treatment is frequently discussed. While chemotherapy is ineffective, it would appear that some molecular targeted therapies, in particular imatinib, could slow down the tumor growth in case-reports, retrospective series, and phase I or II trials. Nineteen publications, between January 1990 and September 2014, have been found describing the activity of these targeted therapies. A systematic analysis of these publications shows that the best objective response with targeted therapies was stabilization in 52 to 69% of chordomas. Given the indolent course of advanced chordoma and because of the absence of randomized trial, the level of evidence to treat chordomas with molecular therapy is low (level III), whatever the drug. Furthermore, we could not draw firm conclusion on the activity of imatinib. Other putative targets have also been described. Therefore, further clinical trials are expected, especially with these targets. Nevertheless, it seems essential, in those future studies, to consider the naturally slow course of the disease. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  11. Sustained Response of a Clivus Chordoma to Erlotinib after Imatinib Failure

    Directory of Open Access Journals (Sweden)

    Aline Houessinon

    2015-01-01

    Full Text Available Chordoma is a rare malignant axial tumour that develops from embryonic remnants of the notochord. Surgery and irradiation are the standard initial treatment. However, local recurrence is frequent and cytotoxic chemotherapy is inefficient. Transient activity of imatinib, a platelet-derived growth factor receptor inhibitor, was described in a phase II study. Activity of epidermal growth factor receptor (EGFR inhibitors (erlotinib, gefitinib has also been shown in a few recent case reports. We describe a 68-year-old female in whom clivus chordoma recurred after surgery and radiotherapy. The tumour progressed despite imatinib treatment. A partial and sustained response (28+ months was obtained using erlotinib, an EGFR inhibitor. Erlotinib should be evaluated in a prospective trial investigating new potential therapies against recurrent chordoma.

  12. Expression study of the target receptor tyrosine kinase of Imatinib mesylate in skull base chordomas.

    Science.gov (United States)

    Orzan, Francesca; Terreni, Maria Rosa; Longoni, Mauro; Boari, Nicola; Mortini, Pietro; Doglioni, Claudio; Riva, Paola

    2007-07-01

    Chordomas are rare neoplasms arising along the axial skeleton. Up to now, the most suitable therapeutic approach is based on a combination of surgical excision and radiotherapy. Chemotherapy in not applied due to its reported low efficacy. Recently, evidence on the efficacy of Imatinib mesylate in two patients has been reported. We analyzed 14 chordoma samples for the expression of the Imatinib mesylate targets by means of RT-PCR and immunohistochemistry and found that PDGFR alpha and PDGFR beta are in some cases expressed in neoplastic cells, while the stromal counterpart of the same tumor shows the above receptors. Findings on the PDGFA/PDGFB expression suggest a receptor-activated status. Our study provides new insights into the specific localization of Imatinib mesylate targets in skull base chordomas that could be taken into account for the setting up of a pharmacological treatment for this tumor.

  13. Sustained response of a clivus chordoma to erlotinib after imatinib failure.

    Science.gov (United States)

    Houessinon, Aline; Boone, Mathieu; Constans, Jean-Marc; Toussaint, Patrick; Chauffert, Bruno

    2015-01-01

    Chordoma is a rare malignant axial tumour that develops from embryonic remnants of the notochord. Surgery and irradiation are the standard initial treatment. However, local recurrence is frequent and cytotoxic chemotherapy is inefficient. Transient activity of imatinib, a platelet-derived growth factor receptor inhibitor, was described in a phase II study. Activity of epidermal growth factor receptor (EGFR) inhibitors (erlotinib, gefitinib) has also been shown in a few recent case reports. We describe a 68-year-old female in whom clivus chordoma recurred after surgery and radiotherapy. The tumour progressed despite imatinib treatment. A partial and sustained response (28+ months) was obtained using erlotinib, an EGFR inhibitor. Erlotinib should be evaluated in a prospective trial investigating new potential therapies against recurrent chordoma.

  14. Optimising the image of the intradural nerve root: the value of MR radiculography

    Energy Technology Data Exchange (ETDEWEB)

    Hofman, P.A.M. [Department of Diagnostic Radiology, University Hospital Maastricht (Netherlands); Wilmink, J.T. [Department of Diagnostic Radiology, University Hospital Maastricht (Netherlands)

    1996-10-01

    We evaluated the additional value of MR radiculography for increasing the sensitivity and specificity of MRI with regard to nerve root compression in patients with sciatica. The single slices of a heavily T 2-weighted oblique coronal image set were reformatted with a maximum intensity projection protocol. This image resembles a classical contrast radiculogram and shows the intradural nerve root and its sleeve. In 43 patients studied with a standard MRI examination there was a need for further assessment of nerve root compression in 19 (44 %). In 13 (68 %) of these, MR radiculography made a definite verdict possible. (orig.). With 4 figs., 2 tabs.

  15. Intradural Procedural Time to Assess Technical Difficulty of Superciliary Keyhole and Pterional Approaches for Unruptured Middle Cerebral Artery Aneurysms.

    Science.gov (United States)

    Choi, Yeon-Ju; Son, Wonsoo; Park, Ki-Su; Park, Jaechan

    2016-11-01

    This study used the intradural procedural time to assess the overall technical difficulty involved in surgically clipping an unruptured middle cerebral artery (MCA) aneurysm via a pterional or superciliary approach. The clinical and radiological variables affecting the intradural procedural time were investigated, and the intradural procedural time compared between a superciliary keyhole approach and a pterional approach. During a 5.5-year period, patients with a single MCA aneurysm were enrolled in this retrospective study. The selection criteria for a superciliary keyhole approach included : 1) maximum diameter of the unruptured MCA aneurysm <15 mm, 2) neck diameter of the MCA aneurysm <10 mm, and 3) aneurysm location involving the sphenoidal or horizontal segment of MCA (M1) segment and MCA bifurcation, excluding aneurysms distal to the MCA genu. Meanwhile, the control comparison group included patients with the same selection criteria as for a superciliary approach, yet who preferred a pterional approach to avoid a postoperative facial wound or due to preoperative skin trouble in the supraorbital area. To determine the variables affecting the intradural procedural time, a multiple regression analysis was performed using such data as the patient age and gender, maximum aneurysm diameter, aneurysm neck diameter, and length of the pre-aneurysm M1 segment. In addition, the intradural procedural times were compared between the superciliary and pterional patient groups, along with the other variables. A total of 160 patients underwent a superciliary (n=124) or pterional (n=36) approach for an unruptured MCA aneurysm. In the multiple regression analysis, an increase in the diameter of the aneurysm neck ( p <0.001) was identified as a statistically significant factor increasing the intradural procedural time. A Pearson correlation analysis also showed a positive correlation (r=0.340) between the neck diameter and the intradural procedural time. When comparing the

  16. Mobile spine chordoma: results of 166 patients from the AOSpine Knowledge Forum Tumor database.

    Science.gov (United States)

    Gokaslan, Ziya L; Zadnik, Patricia L; Sciubba, Daniel M; Germscheid, Niccole; Goodwin, C Rory; Wolinsky, Jean-Paul; Bettegowda, Chetan; Groves, Mari L; Luzzati, Alessandro; Rhines, Laurence D; Fisher, Charles G; Varga, Peter Pal; Dekutoski, Mark B; Clarke, Michelle J; Fehlings, Michael G; Quraishi, Nasir A; Chou, Dean; Reynolds, Jeremy J; Williams, Richard P; Kawahara, Norio; Boriani, Stefano

    2016-04-01

    A chordoma is an indolent primary spinal tumor that has devastating effects on the patient's life. These lesions are chemoresistant, resistant to conventional radiotherapy, and moderately sensitive to proton therapy; however, en bloc resection remains the preferred treatment for optimizing patient outcomes. While multiple small and largely retrospective studies have investigated the outcomes following en bloc resection of chordomas in the sacrum, there have been few large-scale studies on patients with chordomas of the mobile spine. The goal of this study was to review the outcomes of surgically treated patients with mobile spine chordomas at multiple international centers with respect to local recurrence and survival. This multiinstitutional retrospective study collected data between 1988 and 2012 about prognosis-predicting factors, including various clinical characteristics and surgical techniques for mobile spine chordoma. Tumors were classified according to the Enneking principles and analyzed in 2 treatment cohorts: Enneking-appropriate (EA) and Enneking-inappropriate (EI) cohorts. Patients were categorized as EA when the final pathological assessment of the margin matched the Enneking recommendation; otherwise, they were categorized as EI. Descriptive statistics were used to summarize the data (Student t-test, chi-square, and Fisher exact tests). Recurrence and survival data were analyzed using Kaplan-Meier survival curves, log-rank tests, and multivariate Cox proportional hazard modeling. A total of 166 patients (55 female and 111 male patients) with mobile spine chordoma were included. The median patient follow-up was 2.6 years (range 1 day to 22.5 years). Fifty-eight (41%) patients were EA and 84 (59%) patients were EI. The type of biopsy (p mobile spine.

  17. DURABLE RESPONSE OF SPINAL CHORDOMA TO COMBINED INHIBITION OF IGF-1R AND EGFR

    Directory of Open Access Journals (Sweden)

    Tamara eAleksic

    2016-05-01

    Full Text Available Chordomas are rare primary malignant bone tumors arising from embryonal notochord remnants of the axial skeleton. Chordomas commonly recur following surgery and radiotherapy, and there is no effective systemic therapy. Previous studies implicated receptor tyrosine kinases including epidermal growth factor receptor (EGFR and type 1 insulin-like growth factor receptor (IGF-1R in chordoma biology. We report an adult female patient who presented in 2003 with spinal chordoma, treated with surgery and radiotherapy. She underwent further surgery for recurrent chordoma in 2008, with subsequent progression in pelvic deposits. In June 2009 she was recruited onto the Phase I OSI-906-103 trial of EGFR inhibitor erlotinib with linsitinib, a novel inhibitor of IGF-1R/insulin receptor (INSR. Treatment with 100mg QD erlotinib and 50mg QD linsitinib was well-tolerated, and after 18 months a partial response was achieved by RECIST criteria. From 43 months a protocol modification allowed intra-patient linsitinib dose-escalation to 50mg BID. The patient remained stable on trial treatment for a total of five years, discontinuing treatment in August 2014. She subsequently experienced further disease progression for which she underwent pelvic surgery in April 2015. Analysis of DNA extracted from 2008 (pre-trial tissue showed that the tumor harbored wild-type EGFR, and a PIK3CA mutation was detected in plasma but not tumor DNA. The 2015 (post-trial tumor harbored a mutation of uncertain significance in ATM, with no detectable mutations in other components of a 50 gene panel including EGFR, PIK3CA and TP53. By immunohistochemistry the tumor was positive for brachyury, the molecular hallmark of chordoma, and showed weak-moderate membrane and cytoplasmic EGFR. IGF-1R was detected in the plasma membrane and cytoplasm and was expressed more strongly in recurrent tumor than the primary. We also noted heterogeneous nuclear IGF-1R, which has been linked with sensitivity to IGF

  18. Durable Response of Spinal Chordoma to Combined Inhibition of IGF-1R and EGFR.

    Science.gov (United States)

    Aleksic, Tamara; Browning, Lisa; Woodward, Martha; Phillips, Rachel; Page, Suzanne; Henderson, Shirley; Athanasou, Nicholas; Ansorge, Olaf; Whitwell, Duncan; Pratap, Sarah; Hassan, A Bassim; Middleton, Mark R; Macaulay, Valentine M

    2016-01-01

    Chordomas are rare primary malignant bone tumors arising from embryonal notochord remnants of the axial skeleton. Chordomas commonly recur following surgery and radiotherapy, and there is no effective systemic therapy. Previous studies implicated receptor tyrosine kinases, including epidermal growth factor receptor (EGFR) and type 1 insulin-like growth factor receptor (IGF-1R), in chordoma biology. We report an adult female patient who presented in 2003 with spinal chordoma, treated with surgery and radiotherapy. She underwent further surgery for recurrent chordoma in 2008, with subsequent progression in pelvic deposits. In June 2009, she was recruited onto the Phase I OSI-906-103 trial of EGFR inhibitor erlotinib with linsitinib, a novel inhibitor of IGF-1R/insulin receptor (INSR). Treatment with 100 mg QD erlotinib and 50 mg QD linsitinib was well-tolerated, and after 18 months a partial response was achieved by RECIST criteria. From 43 months, a protocol modification allowed intra-patient linsitinib dose escalation to 50 mg BID. The patient remained stable on trial treatment for a total of 5 years, discontinuing treatment in August 2014. She subsequently experienced further disease progression for which she underwent pelvic surgery in April 2015. Analysis of DNA extracted from 2008 (pre-trial) tissue showed that the tumor harbored wild-type EGFR, and a PIK3CA mutation was detected in plasma, but not tumor DNA. The 2015 (post-trial) tumor harbored a mutation of uncertain significance in ATM, with no detectable mutations in other components of a 50 gene panel, including EGFR, PIK3CA, and TP53. By immunohistochemistry, the tumor was positive for brachyury, the molecular hallmark of chordoma, and showed weak-moderate membrane and cytoplasmic EGFR. IGF-1R was detected in the plasma membrane and cytoplasm and was expressed more strongly in recurrent tumor than the primary. We also noted heterogeneous nuclear IGF-1R, which has been linked with sensitivity

  19. Computed tomography and magnetic resonance imaging of thoracic chordoma in a Bengal tiger (Panthera tigris tigris).

    Science.gov (United States)

    Iseri, Toshie; Shimizu, Junichiro; Akiyoshi, Hideo; Kusuda, Kayo; Hayashi, Akiyoshi; Mie, Keiichiro; Izawa, Takeshi; Kuwamura, Mitsuru; Yamate, Jyoji; Fujimoto, Yuka; Ohashi, Fumihito

    2015-07-01

    A Bengal tiger was presented for evaluation of weakness, ataxia and inappetance. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a mass extending from the T7-8 vertebral body to the left rib and compressing the spinal cord. On CT, the bone destruction and sequestrum were shown. On MRI, the multilobulated mass appeared hypo- to isointense in T1-weighted and hyperintense in T2-weighted images. The tiger died after imaging, most likely from renal failure. Chordoma without metastasis was diagnosed on necropsy. The imaging characteristics were similar to those found in chordoma in humans. This report describes the use of CT and MRI in an exotic species.

  20. Gain of chromosome 7 by chromogenic in situ hybridization (CISH) in chordomas is correlated to c-MET expression.

    Science.gov (United States)

    Walter, Beatriz A; Begnami, Maria; Valera, Vladimir A; Santi, Mariarita; Rushing, Elisabeth J; Quezado, Martha

    2011-01-01

    Chordomas are low to intermediate grade malignancies that arise from remnants of embryonic notochord. They often recur after surgery and are highly resistant to conventional adjuvant therapies. Recently, the development of effective targeted molecular therapy has been investigated in chordomas that show receptors for tyrosine kinase (RTKs) activation. Expression of specific RTKs such as Epidermal Growth Factor Receptor (EGFR) and Mesenchymal-epithelial transition factor (c-MET) in chordomas may offer valuable therapeutic options. We investigated changes in copy number of chromosome 7 and correlated it with EGFR gene status and EGFR and c-MET protein expression in 22 chordoma samples. Chromosome 7 copy number was evaluated by chromogenic in situ hybridization (CISH) and protein expression of EGFR and c-MET by immunohistochemistry. Tumors mostly showed conventional histopathologic features and were found mainly in sacral (41%) and cranial sites (54.5%). Aneusomy of chromosome 7 was seen in 73% of the samples, 62% of primary tumors and in all recurrent chordomas. EGFR and c-MET were both expressed, but only c-MET protein expression was significantly correlated with chromosome 7 aneusomy (P ≤ 0.001). c-MET overexpression may represent an early chromosome 7 alteration that could play an important role during chordoma pathogenesis. c-MET overexpression shows promise as a molecular marker of response to targeted molecular therapy in the treatment of chordomas.

  1. Immunohistochemical expression of receptor tyrosine kinase PDGFR-α, c-Met, and EGFR in skull base chordoma.

    Science.gov (United States)

    Akhavan-Sigari, R; Abili, M; Gaab, M R; Rohde, V; Zafar, N; Emami, P; Ostertag, H

    2015-01-01

    Chordomas are rare, locally aggressive malignancies that often exhibit an insidious natural history and are difficult to eradicate. Surgery and radiotherapy are the treatment mainstays of chordoma, but the chance of local recurrence remains high. Reports of receptor tyrosine kinase (RTK) expression in chordoma suggest that these tumors may respond to kinase inhibitor therapy. Currently, there are no effective chemotherapeutic protocols for chordoma. A tissue microarray containing 74 tumor specimens from primary chordoma patients and 71 from their recurrent tumors for a total of 145 tumor specimens was immunohistochemically analyzed for expression of a number of proteins involved in signal transduction from RTKs. Platelet-derived growth factor receptor-α (PDGFR-α), epidermal growth factor receptor (EGFR), c-Met, and CD-34 were detected in 100, 92, 100, and 59% of cases, respectively. PDGFR-α and c-Met staining was of moderate to strong intensity in all cases. In contrast, total EGFR staining was variable; weak staining was detected in 10 cases. Our results contribute to the understanding of the expression of RTKs in skull base chordomas and support the development of targeted therapies that inhibit RTKs, which may have a synergistic effect for chemotherapy in patients. There were statistically significant correlations between the expression of PDGFR-α, c-Met, and EGFR and disease-free survival. The results nonetheless suggest that chordomas may respond to RTK inhibitors or modulators of other downstream signaling.

  2. Spinal intradural hydatid cyst causing arachnoiditis: A rare etiology of cauda equina syndrome

    Directory of Open Access Journals (Sweden)

    Suyash Singh

    2016-01-01

    Full Text Available This study aims to focus on a rare presentation of spinal hydatid cyst as cauda equine syndrome and misdiagnosed as intradural extramedullary (IDEM benign lesion on magnetic resonance imaging. In this article, we report a case of spinal hydatid cyst masquerading as IDEM tumor, and intraoperatively, we accidently find clumped granuloma with severe arachnoiditis and hydatid cyst in lumber region, which was present as bilateral S1 radiculopathy with cauda equina syndrome. An 11 year old boy who presented with symptoms and signs of cauda equina syndrome and planned for surgical excision. His radiological impression was IDEM possibly neurofibroma. To our surprise, we found multiple intradural cystic lesions with arachnoiditis. Dissecting in plane cyst was flushed out, and surgical cavity was irrigated with 3% saline. Postoperatively histopathology and serum tests confirmed the diagnosis of hydatid cyst. Hydatid disease is rare cause of cauda equine syndrome which can be miss diagnosed on radiological investigations. A high index of suspicion should be kept especially in a young patient from the Indian subcontinent.

  3. Surgery for ventral intradural thoracic spinal tumors with a posterolateral transpedicular approach.

    Science.gov (United States)

    Ito, Kiyoshi; Aoyama, Tatsuro; Miyaoka, Yoshinari; Seguchi, Tatsuya; Horiuchi, Tetsuyoshi; Hongo, Kazuhiro

    2016-08-01

    Surgery for ventrally seated thoracic tumors requires an anatomically specific approach that is distinct from cervical or lumbar spinal cord surgery as the narrower spinal canal of the thoracic spinal cord makes it sensitive to surgical procedures. However, reports describing this operative technique are few. To obtain a wide operative field and minimize thoracic spinal cord retraction, we employed a posterolateral transpedicular approach in ventral-located tumors and investigated the efficacy and limitations of this technique. Eighteen patients with lesions (meningioma or neurinoma) located in the ventral intradural thoracic region were surgically treated between 2009 and 2014. The relationship among the clinical outcome, tumor location, and postoperative spinal alignment was analyzed. Postoperative neurological function improved in all patients, namely those with meningioma (p = 0.012) and schwannoma (p = 0.018). One patient who underwent removal of two facet joints suffered a postoperative compression fracture. Removal of two facet joints and pedicles resulted in a worsening of spinal alignment (p = 0.03), while this was not the case for the removal of one facet joint and pedicle (p = 0.72). This case series clarified the benefits of the posterolateral transpedicular approach for resection of ventral intradural extramedullary tumors. Removal of one pedicle and facet joint seems to be more beneficial.

  4. [Neurosurgical Outcomes of Intradural Extramedullary Spinal Tumors in 97 cases:Siberian Experience].

    Science.gov (United States)

    Byvaltsev, Vadim Anatolyevich; Damdinov, Bair Batiyevich; Belykh, Evgenii Georgiyevich; Ivanova, Kristina Olegovna; Sorokovikov, Vladimir Alekseevich; Tsunetoshi, Kenzo; Kitai, Ryuhei; Kikuta, Kenichiro

    2017-09-01

    We retrospectively investigated the clinical presentation and outcome of patients with spinal intradural extramedullary tumors, which had been surgically treated. A total of 97 consecutive patients(32 males and 65 females;mean age, 48 years;range, 17-79 years)underwent surgery between 2004 and 2014 at Irkutsk State Medical Academy and affiliated hospitals. To determine presentation and outcomes associated with these tumors, we evaluated the waiting period before surgery, neurological symptoms including motor, sensory, urinary, and rectal dysfunction, modified McCormick scale, Macnab's outcome assessment of patient satisfaction, surgical procedure, tumor location, and histological diagnosis. The most frequent preoperative symptom was myelopathy(52.6%)at presentation, followed by radiculopathy(20.6%), and a combination of both(26.8%). Surgical intervention produced highly successful results, in which 77 of 97 cases showed neurological improvement. However, the symptoms were not completely reversed, and there was improvement only the equivalent to one grade in the modified McCormick scale for most patients. Surgical outcomes were not influenced by tumor location, extent of lamina resection, or histological diagnosis, but there was a correlation between greater patient improvement and a shorter waiting period before surgery. As such, we recommend early total resection for symptomatic spinal intradural extramedullary tumors.

  5. Chordomas of the Skull Base, Mobile Spine, and Sacrum: An Epidemiologic Investigation of Presentation, Treatment, and Survival.

    Science.gov (United States)

    Zuckerman, Scott L; Bilsky, Mark H; Laufer, Ilya

    2018-02-25

    Chordomas are rare primary bone tumors that arise from the axial skeleton. Our objective was to analyze trends in radiation and surgery over time and determine location-based survival predictors for chordomas of the skull base, mobile spine, and sacrum. A retrospective cohort study of the SEER (Surveillance Epidemiology and End Results) database from 1973 to 2013 was conducted. All patients had histologically confirmed chordomas. The principal outcome measure was overall survival (OS). The cohort included 1616 patients: skull base (664), mobile spine (444), and sacrum (508). Skull base tumors presented earliest in life (47.4 years) and sacral tumors presented latest (62.7 years). Rates of radiation remained stable for skull base and mobile spine tumors but declined for sacral tumors (P = 0.006). Rates of surgical resection remained stable for skull base and sacral tumors but declined for mobile spine tumors (P = 0.046). Skull base chordomas had the longest median survival (162 months) compared with mobile spine (94 months) and sacral tumors (87 months). Being married was independently associated with improved OS for skull base tumors (hazard ratio, 0.73; 95% confidence interval, 0.53-0.99; P = 0.044). Surgical resection was independently associated with improved OS for sacral chordomas (hazard ratio, 0.48; 95% confidence interval, 0.34-0.69; P mobile spine chordomas and radiation for sacral chordomas decreased over time. Patients with skull base tumors survived longer than did patients with mobile spine and sacral chordomas, and surgical resection was associated with improved survival in sacral chordomas only. Understanding the behavior of these tumors can help cranial and spinal surgeons improve treatment in this patient population. Copyright © 2018 Elsevier Inc. All rights reserved.

  6. A rare case of concomitant cervical disc herniation and intradural meningioma treated with one-stage posterior surgery.

    Science.gov (United States)

    Hu, Xiaojian; Chen, Zhong; Wang, Yue

    2017-12-14

    Case report. To present a rare case of cervical disc herniation concomitant with intradural meningioma which was successfully treated using a single one-stage posterior surgery of tumor resection and transdural discectomy. Coexistence of symptomatic disc herniation and intra-spinal tumor in the same cervical segment is extremely rare. Usually, two-stage anterior and posterior surgeries are needed to treat two conditions, respectively. One-stage posterior surgery to treat two pathologies simultaneously has not been reported in the literature. A 76-year-old man presented with leg weakness and numbness for 6 months and left arm pain for 2 months. Contrast MR imaging revealed C3/4 intervertebral disc herniation and a hyperintense intradural lesion at the right portion of C3 canal. A one-stage posterior surgery, including C3/4 laminectomy, intradural tumor resection, transdural C3/4 discectomy, and C3/4 lateral mass instrumentation and fusion, was performed to treat two distinct pathologies together. The patient's arm pain and numbness disappeared right after the surgery and symptoms of myelopathy fully recovered at 6-month follow-up. Histological studies confirmed a herniated disc and a meningioma. In rare case, intradural tumor coexists with cervical disc herniation. When suspicious findings were noticed, or clinical symptoms cannot be fully explained, contrast MR imaging is helpful in differential diagnosis. Microscopic transdural discectomy is safe, and could be used as an optional procedure for cervical disc herniation in some cases.

  7. Endoscopic laser ablation of clival chordoma with magnetic resonance-guided laser induced thermal therapy

    Directory of Open Access Journals (Sweden)

    James Barrese

    2014-12-01

    Conclusion: The endoscopic endonasal approach to MRI-guided laser ablation is both technically feasible and safe. As a result, this therapy may be a useful alternative in hard-to-reach chordomas, or in recurrent cases that have failed other conventional treatment modalities.

  8. Distribution of Age and Location of Chordoma in 39 Cases and Review of Treatment Options

    Directory of Open Access Journals (Sweden)

    Shahab Kamali Ardekani

    2012-02-01

    Full Text Available Introduction: notochord. Although histologically benign, these tumors are locally aggressiveand present significant managment challenges . There arew some studies onevaluated the location, age and gender of the patients with Chordoma in tworeferral centers in Tehran.chordoma cases but there was no study about Iranian cases. In this study weSkull base chordomas are rare neoplasms arising from theMethods: (Shariati and Imam Hospitals, Tehran from 2001 to 2011 was retrospectivelyreviewed.A database of patients with chordoma tumors referred to two centersResults: women, and they are most commonly diagnosed in middle-aged (mean age was50.6. Tumors typically occur in the axial skeleton and have a tendency for thespheno-occipital region of the skull base and sacral region. In adults 33.3% ofchordomas involve the sacrococcygeal region, 53% occured at the base of theskull near the spheno-occipital area, and near 14% were found in the vertebralcolumn. The cranial nerves mostly affected were abducens, oculomotor andtrochlear, with some overlaps. All patients were treated with surgery and somecases referred for gamma-knife radiosurgery (GKS.In our subjects tumors affect men nearly twice as frequently asDiscussion: to females that is different from other studies, however, few studies reportedmore male to female ratio. Despite the progress in current surgical techniquesand some encouraging results with the use of targeted therapy, disease controland long-term prognosis of patients are still poor.Findings of this study showed more involvement of males compare

  9. Adult-onset intradural spinal teratoma: report of 18 consecutive cases and outcomes in a single center.

    Science.gov (United States)

    Wan, Wei; Yang, Cheng; Yan, Wangjun; Liu, Tielong; Yang, Xinghai; Song, Dianwen; Xiao, Jianru

    2017-07-01

    Eighteen consecutive patients with adult-onset intradural spinal teratoma underwent surgical treatment in our center from 1998 to 2013. Teratoma is defined as a neoplasm composed of elements derived from three germ cell layers (ectoderm, endoderm and mesoderm). Intraspinal teratoma is extremely rare and accounts for 0.2-0.5% of all spinal cord tumors. Moreover, teratoma occurs primarily in neonates and young children. Adult-onset intradural spinal teratoma is even rare. The aim of this study was to discuss the clinical characteristics, diagnosis and therapeutic strategies of adult-onset intradural spinal teratoma. This retrospective study included 18 consecutive adult patients with intradural teratoma who were surgically treated in our center between 1998 and 2013. The clinical features, pathogenesis, diagnostic strategies and surgical outcomes were discussed. Neurological function outcomes were evaluated by the JOA scoring system. Of the 18 included patients, 4 patients received subtotal resection and the other 14 patients received total resection. All the 18 cases were diagnosed with mature teratoma. The mean follow-up period was 79.7 (median 60.5; range 27-208) months. Local recurrence occurred in two of the four patients who underwent subtotal resection and in no patient who underwent total resection. The neurologic status improved in 16 cases and remained unchanged in the other two patients. Adult-onset intradural spinal teratoma is extremely rare. To the best of our knowledge, this is the largest series of patients with this disease. Despite the slow-growth and indolent nature, radical resection remains the recommended treatment to reduce tumor recurrence.

  10. Single-Fraction Spine Stereotactic Body Radiation Therapy for the Treatment of Chordoma.

    Science.gov (United States)

    Jung, Edward W; Jung, David L; Balagamwala, Ehsan H; Angelov, Lilyana; Suh, John H; Djemil, Toufik; Magnelli, Anthony; Chao, Samuel T

    2017-06-01

    Chordoma is a radioresistant tumor that presents a therapeutic challenge with spine involvement, as high doses of radiation are needed for local control while limiting dose to the spinal cord. The purpose of this study is to determine the efficacy and safety of single-fraction spine stereotactic body radiation therapy for the treatment of spine chordoma. A retrospective review of our institutional database from 2006 to 2013 identified 8 patients (12 cases) with chordoma of the spine who were treated with spine stereotactic body radiation therapy. Surgical resection was performed in 7 of the 12 cases. The treatment volume was defined by the bony vertebral level of the tumor along with soft tissue extension appreciated on magnetic resonance imaging fusion. Medical records and imaging were assessed for pain relief and local control. Treatment toxicity was evaluated using Common Terminology Criteria for Adverse Events version 4.0. Median age was 59 years (range, 17-91). Median target volume was 48 cm 3 (1-304), and median prescription dose was 16 Gy (11-16). Median conformality index was 1.44 (1.14-3.21), and homogeneity index was 1.12 (1.05-1.19). With a median follow-up time of 9.7 months (.5-84), local control was achieved in 75% of the cases treated. One patient developed limited grade 2 spinal cord myelopathy that resolved with steroids. There were no other treatment toxicities from spine stereotactic body radiation therapy. Single-fraction spine stereotactic body radiation therapy can be safely delivered to treat chordoma of the spine with the potential to improve pain symptoms. Although the early data are suggestive, long-term follow-up with more patients is necessary to determine the efficacy of spine stereotactic body radiation therapy in the treatment of chordoma of the spine.

  11. Predicting clinical outcomes in chordoma patients receiving immunotherapy: a comparison between volumetric segmentation and RECIST

    International Nuclear Information System (INIS)

    Fenerty, Kathleen E.; Folio, Les R.; Patronas, Nicholas J.; Marté, Jennifer L.; Gulley, James L.; Heery, Christopher R.

    2016-01-01

    The Response Evaluation Criteria in Solid Tumors (RECIST) are the current standard for evaluating disease progression or therapy response in patients with solid tumors. RECIST 1.1 calls for axial, longest-diameter (or perpendicular short axis of lymph nodes) measurements of a maximum of five tumors, which limits clinicians’ ability to adequately measure disease burden, especially in patients with irregularly shaped tumors. This is especially problematic in chordoma, a disease for which RECIST does not always adequately capture disease burden because chordoma tumors are typically irregularly shaped and slow-growing. Furthermore, primary chordoma tumors tend to be adjacent to vital structures in the skull or sacrum that, when compressed, lead to significant clinical consequences. Volumetric segmentation is a newer technology that allows tumor burden to be measured in three dimensions on either MR or CT. Here, we compared the ability of RECIST measurements and tumor volumes to predict clinical outcomes in a cohort of 21 chordoma patients receiving immunotherapy. There was a significant difference in radiologic time to progression Kaplan-Meier curves between clinical outcome groups using volumetric segmentation (P = 0.012) but not RECIST (P = 0.38). In several cases, changes in volume were earlier and more sensitive reflections of clinical status. RECIST is a useful evaluation method when obvious changes are occurring in patients with chordoma. However, in many cases, RECIST does not detect small changes, and volumetric assessment was capable of detecting changes and predicting clinical outcome earlier than RECIST. Although this study was small and retrospective, we believe our results warrant further research in this area

  12. Efficacy of pazopanib and sunitinib in advanced axial chordoma: a single reference centre case series.

    Science.gov (United States)

    Lipplaa, Astrid; Dijkstra, Sander; Gelderblom, Hans

    2016-01-01

    Chordomas are rare malignant tumours of the axial skeleton and skull base supposed to arise from cellular remnants of the notochord. These tumours have the potential to metastasize (30-40 %), usually in the later course of the disease. However, the greatest morbidity is usually a result of loco-regional recurrence with infiltration and destruction of surrounding bone and soft tissue. Patients with unresectable or metastatic chordoma are faced with a poor prognosis since cytotoxic chemotherapy or other systemic therapies have not proven their efficacy yet. However, several molecularly targeted drugs have been proposed as potentially beneficial, including tyrosine kinase inhibitors (TKIs) directed at vascular endothelial growth factor receptor (VEGFR), like pazopanib and sunitinib. Five patients with unresectable or metastatic chordoma were treated with VEGFR inhibitors pazopanib or sunitinib in the Leiden University Medical Centre (LUMC) between 2008 and 2015. Two out of four patients treated with pazopanib derived clinical benefit and disease remained stable for respectively 14 and 15 months. The one patient treated with sunitinib achieved a partial response according to RECIST 1.1 which lasted for a total of 27 months. No serious adverse events were observed. These results on the use of pazopanib and sunitinib in chordoma are promising, with an objective response on sunitinib and a median progression free interval of 8.5 months (range 3-15 months), comparable to that of imatinib, in the pazopanib subgroup. However further research is needed to assess the definite role of VEGFR inhibitors in chordoma.

  13. Afatinib is a new therapeutic approach in chordoma with a unique ability to target EGFR and Brachyury.

    Science.gov (United States)

    Magnaghi, Paola; Salom, Barbara; Cozzi, Liviana; Amboldi, Nadia; Ballinari, Dario; Tamborini, Elena; Gasparri, Fabio; Montagnoli, Alessia; Raddrizzani, Laura; Somaschini, Alessio; Bosotti, Roberta; Orrenius, Christian; Bozzi, Fabio; Pilotti, Silvana; Galvani, Arturo; Sommer, Josh; Stacchiotti, Silvia; Isacchi, Antonella

    2017-12-13

    Chordomas are rare bone tumors with no approved therapy. These tumors express several activated tyrosine kinase receptors, which prompted attempts to treat patients with tyrosine kinase inhibitors. Although clinical benefit was observed in Phase II clinical trials with imatinib and sorafenib, and sporadically also with EGFR inhibitors, therapies evaluated to date have shown modest activity. With the goal of identifying new drugs with immediate therapeutic potential for chordoma patients, we collected clinically approved drugs and other advanced inhibitors of MET, PDGFRβ and EGFR tyrosine kinases, and assessed their anti-proliferative activity against a panel of chordoma cell lines. Chordoma cell lines were not responsive to MET and PDGFRβ inhibitors. U-CH1 and UM-Chor1 were sensitive to all EGFR inhibitors, while the remaining cell lines were generally insensitive to these drugs. Afatinib was the only EGFR inhibitor with activity across the chordoma panel. We then investigated the molecular mechanisms behind the responses observed and found that the anti-proliferative IC50s correlate with the unique ability of afatinib to promote degradation of EGFR and brachyury, an embryonic transcription factor considered a key driver of chordoma. Afatinib displayed potent antitumor efficacy in U-CH1, SF8894, CF322 and CF365 chordoma tumor models in vivo. In the panel analyzed, high EGFR phosphorylation and low AXL and STK33 expression correlated with higher sensitivity to afatinib and deserve further investigation as potential biomarkers of response. These data support the use of afatinib in clinical trials and provide the rationale for the upcoming European phase II study on afatinib in advanced chordoma. Copyright ©2017, American Association for Cancer Research.

  14. Large intradural craniospinal arachnoid cyst: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Souvagya Panigrahi

    2012-01-01

    Full Text Available Presence of an arachnoid cyst at craniospinal junction is not very common. This is a very rare anatomic site, with only seven other cases reported in the literature. We report a case of large intradural craniospinal arachnoid cyst presenting with obstructive hydrocephalus and cranial nerve palsy. A 39-year-old male presented with 8-month history of neck pain, headache, vomiting, visual disturbances, diminished taste sensation, and numbness of face. He had bilateral papilledema on ophthalmoscopy. Magnetic resonance imaging (MRI revealed a posterior fossa arachnoid cyst extending down to the lower border of C5 vertebra. Posterior decompression was done through C5 laminectomy. He made a full recovery and was asymptomatic at 6-month follow-up examination. The clinical features, diagnosis, and management of these rare craniospinal arachnoid cysts are discussed.

  15. Back pain in patients with degenerative spine disease and intradural spinal tumor: what to treat? when to treat?

    Science.gov (United States)

    Bellut, David; Mutter, Urs M; Sutter, Martin; Eggspuehler, Andreas; Mannion, Anne F; Porchet, François

    2014-04-01

    Back pain is common in industrialized countries and one of the most frequent causes of work incapacity. Successful treatment is, therefore, not only important for improving the symptoms and the quality of life of these patients but also for socioeconomic reasons. Back pain is frequently caused by degenerative spine disease. Intradural spinal tumors are rare with an annual incidence of 2-4/1,00,000 and are mostly associated with neurological deficits and radicular and nocturnal pain. Back pain is not commonly described as a concomitant symptom, such that in patients with both a tumor and degenerative spine disease, any back pain is typically attributed to the degeneration rather than the tumor. The aim of the present retrospective investigation was to study and analyze the impact of microsurgery on back/neck pain in patients with intradural spinal tumor in the presence of degenerative spinal disease in adjacent spinal segments. Fifty-eight consecutive patients underwent microsurgical, intradural tumor surgery using a standardized protocol assisted by multimodal intraoperative neuromonitoring. Clinical symptoms, complications and surgery characteristics were documented. Standardized questionnaires were used to measure outcome from the surgeon's and the patient's perspectives (Spine Tango Registry and Core Outcome Measures Index). Follow-up included clinical and neuroradiological examinations 6 weeks, 3 months and 1 year postoperatively. Back/neck pain as a leading symptom and coexisting degenerative spine disease was present in 27/58 (47 %) of the tumor patients, and these comprised to group under study. Patients underwent tumor surgery only, without addressing the degenerative spinal disease. Remission rate after tumor removal was 85 %. There were no major surgical complications. Back/neck pain as the leading symptom was eradicated in 67 % of patients. There were 7 % of patients who required further invasive therapy for their degenerative spinal disease. Intradural

  16. Intradural pathology and pathophysiology associated with Chiari I malformation in children and adults with and without syringomyelia.

    Science.gov (United States)

    Dlouhy, Brian J; Dawson, Jeffrey D; Menezes, Arnold H

    2017-12-01

    OBJECTIVE The pathophysiology underlying tonsillar herniation and CSF obstruction in Chiari malformation Type I (CM-I) is unclear, and the cause of CM-I-associated syringomyelia is not well understood. A better understanding of this pathophysiology is important for an improved treatment strategy. Therefore, the authors sought to identify, characterize, and examine the intradural pathology and CSF flow pathophysiology in the posterior fossa and at the level of the foramen magnum that occurs in the setting of CM-I. They determined the incidence of these intradural findings and assessed differences across age, with the degree of tonsillar herniation, and in the presence and absence of syringomyelia. METHODS A prospective database initiated in March 2003 recorded all intraoperative findings during surgical treatment of children and adults with CM-I with or without syringomyelia. A total of 389 surgeries for CM-I were performed in 379 patients between March 2003 and June 2016. A total of 109 surgeries were performed in 109 patients with CM-I (without osseoligamentous abnormalities) in whom both a posterior fossa extradural and intradural decompression with duraplasty was performed (first-time intradural procedures). Using a surgical microscope, intradural pathology and obstruction of CSF channels were identified and assessed. Student t-tests and Fisher's exact tests compared groups in a series of univariate analyses, followed by multivariate logistic regression. RESULTS The following intradural pathological entities were observed (prevalence noted in parentheses). These include those that did not obstruct CSF flow channels: opacified arachnoid (33.0%), thickened arachnoid (3.7%), ischemic and gliotic tonsils (40.4%), tonsillar cysts (0.9%), and inferior descent of the fourth ventricle and cervicomedullary junction (CMJ) (78.0%). The following intradural pathological entities were observed to obstruct CSF flow channels: medialized tonsils (100%), tonsil overlying and

  17. Image-guided percutaneous lipiodol-pingyangmycin suspension injection therapy for sacral chordoma

    International Nuclear Information System (INIS)

    Huang, Dexiao; Chen, Yong; Zeng, Qingie; Li, Yanhao; Wu, Renhua

    2013-01-01

    A 74-year-old man presented with a progressively worsening pain in sacrum and was diagnosed to have a sacral chordoma by biopsy in May, 2004. Percutaneous intratumoral injection with lipiodol-pingyangmycin suspension (LPS) was carried out under image guidance and repeated when the pain in sacrum recurred and the tumor increased. During a 6-year follow-up period, three sessions of this treatment were executed. CT imaging and Karnofsky Performance Score were used to evaluate the size of tumor and quality of life, respectively. The patient was free of pain after each procedure and had a high quality of life with a Karnofsky Performance Score above 80 points. The tumor lesion in sacral area was effectively controlled. No complications were observed. Percutaneous intratumoral injection with LPS under image guidance may be an effective and safe alternative for the patients with sacral chordoma.

  18. Cordoma Sacrococcígeo gigante: relato de caso Giant Sacrococcygeal chordoma: case report

    Directory of Open Access Journals (Sweden)

    Tiago Leal Ghezzi

    2009-06-01

    Full Text Available Cordoma sacrococcígeo é uma neoplasia maligna rara que se origina de remanescentes da notocorda. A localização crítica, comportamento localmente agressivo, reconhecida resistência à radioterapia, significativa morbimortalidade cirúrgica e elevada taxa de recidiva tornam seu tratamento um desafio. Descrevemos um caso de cordoma sacrococcígeo gigante.Sacrococcygeal chordoma is a rare malignant neoplasm arised from the remmants of the notochord. The critical localization, locally aggressive behavior, well-known resistance to radiation therapy, meaningful surgical morbimortality and increased recurrence rate become its treatment a challenge. We describe a case of a giant unresectable sacrococcygeal chordoma.

  19. Focal Anterior Displacement of the Thoracic Spinal Cord without Evidence of Spinal Cord Herniation or an Intradural Mass

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jong Yoon; Lee, Joon Woo; Lee, Guen Young; Kang, Heung Sik [Department of Radiology, Seoul National University Bundang Hospital, Seongnam 463-707 (Korea, Republic of)

    2014-07-01

    We report magnetic resonance imaging (MRI) findings on focal anterior displacement of the thoracic spinal cord in asymptomatic patients without a spinal cord herniation or intradural mass. We identified 12 patients (male:female = 6:6; mean age, 51.7; range, 15-83 years) between 2007 and 2011, with focal anterior displacement of the spinal cord and without evidence of an intradural mass or spinal cord herniation. Two radiologists retrospectively reviewed the MRI findings in consensus. An asymmetric spinal cord deformity with a focal dented appearance was seen on the posterior surface of the spinal cord in all patients, and it involved a length of 1 or 2 vertebral segments in the upper thoracic spine (thoracic vertebrae 1-6). Moreover, a focal widening of the posterior subarachnoid space was also observed in all cases. None of the patients had myelopathy symptoms, and they showed no focal T2-hyperintensity in the spinal cord with the exception of one patient. In addition, cerebrospinal fluid (CSF) flow artifacts were seen in the posterior subarachnoid space of the affected spinal cord level. Computed tomography myelography revealed preserved CSF flow in the two available patients. Focal anterior spinal cord indentation can be found in the upper thoracic level of asymptomatic patients without a spinal cord herniation or intradural mass.

  20. Focal Anterior Displacement of the Thoracic Spinal Cord without Evidence of Spinal Cord Herniation or an Intradural Mass

    International Nuclear Information System (INIS)

    Lee, Jong Yoon; Lee, Joon Woo; Lee, Guen Young; Kang, Heung Sik

    2014-01-01

    We report magnetic resonance imaging (MRI) findings on focal anterior displacement of the thoracic spinal cord in asymptomatic patients without a spinal cord herniation or intradural mass. We identified 12 patients (male:female = 6:6; mean age, 51.7; range, 15-83 years) between 2007 and 2011, with focal anterior displacement of the spinal cord and without evidence of an intradural mass or spinal cord herniation. Two radiologists retrospectively reviewed the MRI findings in consensus. An asymmetric spinal cord deformity with a focal dented appearance was seen on the posterior surface of the spinal cord in all patients, and it involved a length of 1 or 2 vertebral segments in the upper thoracic spine (thoracic vertebrae 1-6). Moreover, a focal widening of the posterior subarachnoid space was also observed in all cases. None of the patients had myelopathy symptoms, and they showed no focal T2-hyperintensity in the spinal cord with the exception of one patient. In addition, cerebrospinal fluid (CSF) flow artifacts were seen in the posterior subarachnoid space of the affected spinal cord level. Computed tomography myelography revealed preserved CSF flow in the two available patients. Focal anterior spinal cord indentation can be found in the upper thoracic level of asymptomatic patients without a spinal cord herniation or intradural mass

  1. Management and outcome of chordomas in the pediatric population: The Hospital for Sick Children experience and review of the literature.

    Science.gov (United States)

    Tsitouras, Vassilios; Wang, Shelly; Dirks, Peter; Drake, James; Bouffet, Eric; Hawkins, Cynthia; Laughlin, Suzanne; Rutka, James T

    2016-12-01

    Chordomas are tumors arising from remnants of the embryological notochord, most commonly found in the spheno-occipital, spinal, or sacro-coccygeal areas. They are rare tumors in the pediatric population and are challenging to manage due to their difficult accessibility, proximity to important anatomy and extension into adjacent structures. We report a series of 10 children treated for chordoma at The Hospital for Sick Children focusing on their surgery, adjuvant therapy and long-term outcomes. A retrospective review involving patient charts, radiographic imaging, and pathology slides was performed for 10 chordoma patients during the period from 1987-2015. Important variables, including patient demographics, chordoma location, presentation, imaging characteristics, pathology subtype, treatment options, and long-term outcome were analysed. The series consists of seven girls and three boys with cranial or upper cervical spine chordomas. One patient presented with an extradural left cerebellopontine angle chordoma demonstrating aggressive and dedifferentiated features, which, to our knowledge, has not been previously described in the literature. All patients received surgical resection followed by photon or proton radiotherapy. Four patients with chondroid or atypical pathology also received chemotherapeutic adjuvants. All patients with classical pathology achieved favourable outcome, while the four patients with atypical pathology progressed quickly despite aggressive therapy, suggesting that pathology subtype is a crucial prognostic factor. This study summarizes 30years of surgical and adjuvant therapy experience in a large academic center for pediatric chordoma patients. Patient outcomes were dependent on pathology subtype, and a multidisciplinary approach involving surgery, radiotherapy, and chemotherapy can be considered on an individual basis. Copyright © 2016 Elsevier Ltd. All rights reserved.

  2. Sustained Response of a Clivus Chordoma to Erlotinib after Imatinib Failure

    OpenAIRE

    Aline Houessinon; Mathieu Boone; Jean-Marc Constans; Patrick Toussaint; Bruno Chauffert

    2015-01-01

    Chordoma is a rare malignant axial tumour that develops from embryonic remnants of the notochord. Surgery and irradiation are the standard initial treatment. However, local recurrence is frequent and cytotoxic chemotherapy is inefficient. Transient activity of imatinib, a platelet-derived growth factor receptor inhibitor, was described in a phase II study. Activity of epidermal growth factor receptor (EGFR) inhibitors (erlotinib, gefitinib) has also been shown in a few recent case reports. We...

  3. Cordoma sacrococcígeo gigante - Relato de caso=Giant sacrococcygeal chordoma - Case report

    OpenAIRE

    Ghezzi, Tiago Leal; Pereira Filho, Gustavo Azambuja; Cigerza, Giuliano Chemale; Cruz, Dennis Baroni; Pêgas, Karla Lais; Corleta, Oly Campos

    2009-01-01

    Cordoma sacrococcígeo é uma neoplasia maligna rara que se origina de remanescentes da notocorda. A localização crítica, comportamento localmente agressivo, reconhecida resistência à radioterapia, significativa morbimortalidade cirúrgica e elevada taxa de recidiva tornam seu tratamento um desafio. Descrevemos um caso de cordoma sacrococcígeo gigante. Sacrococcygeal chordoma is a rare malignant neoplasm arised from the remmants of the notochord. The critical localization, locally aggressive ...

  4. Cordoma Sacrococcígeo gigante: relato de caso Giant Sacrococcygeal chordoma: case report

    OpenAIRE

    Tiago Leal Ghezzi; Gustavo Pereira Filho; Giuliano Chemale Cigerza; Oly Campos Corleta

    2009-01-01

    Cordoma sacrococcígeo é uma neoplasia maligna rara que se origina de remanescentes da notocorda. A localização crítica, comportamento localmente agressivo, reconhecida resistência à radioterapia, significativa morbimortalidade cirúrgica e elevada taxa de recidiva tornam seu tratamento um desafio. Descrevemos um caso de cordoma sacrococcígeo gigante.Sacrococcygeal chordoma is a rare malignant neoplasm arised from the remmants of the notochord. The critical localization, locally aggressive beha...

  5. Cordoma de ápice petroso: relato de um caso Petrous apex chordoma: a case report

    Directory of Open Access Journals (Sweden)

    Cláudio Régis S. Silveira

    2001-02-01

    Full Text Available Cordomas são neoplasias raras que se originam dos remanescentes da notocorda primitiva. Estes remanescentes persistem ao longo de todo o esqueleto axial. Os cordomas intracranianos, mais freqüentemente, se localizam no clivus, próximo à sincondrose esfenooccipital, tipicamente na linha média. Nós descrevemos um caso atípico de cordoma fora da linha média, mais especificamente no ápice petroso, e discutimos as causas embriológicas que determinam esta localização, bem como sintomas, achados de imagem, tratamento cirúrgico e evolução.Chordomas are rare neoplasms arising from notochordal remnants that persist along the axial skeleton. Intracranial chordomas occur more frequently in the midline. We describe an atypical case of an off-midline chordoma arising from the petrous apex, and discuss the embryogenic factors which determine that location, as well as the symptoms, imaging findings, surgical treatment and evolution.

  6. Minimally invasive approach for small ventrally located intradural lesions of the craniovertebral junction.

    Science.gov (United States)

    Eicker, Sven O; Mende, Klaus Christian; Dührsen, Lasse; Schmidt, Nils Ole

    2015-04-01

    The surgical management of lesions ventral to the neuraxis at the level of the craniovertebral junction (CVJ) and upper cervical spine is challenging. Here, the authors describe a minimally invasive dorsal approach for small ventrally located intradural lesions at the CVJ as an alternative for the more extensive classic transoral approach or variants of suboccipital approaches. Between 2012 and 2014, 6 symptomatic patients with a small lesion of the ventral aspect at the CVJ level were treated using a minimally invasive dorsal approach at the University Medical Center in Hamburg-Eppendorf, Germany. The anatomical distance between the posterior atlantooccipital membrane and the posterior atlantoaxial ligament, as determined by CT images, was assessed in the treated patients and in 100 untreated persons. The authors treated 6 patients (mean age 54.7 years) who had a clinical presentation of mild neurological symptoms that disappeared after resection. Minimally invasive surgical dorsal access was achieved by using tubular systems and using the natural space between the occiput (C-0) and C-1, and in 1 case between C-1 and C-2, without having to remove bony structures. The postoperative course in each of the 6 patients was uneventful. The neuropatho-logical findings confirmed a meningotheliomatous meningioma (WHO Grade I) in 5 cases and an extramedullary cavernous hemangioma in 1 case. MRI confirmed complete resection of all the lesions. The atlantooccipital distances ranged from 3 to 17 mm (mean 8.98 mm) in the supine neutral position, and the atlantoaxial distances ranged from 5 to 17 mm (mean 10.56 mm). There were no significant differences between women and men (atlantooccipital p = 0.14; atlantoaxial p = 0.72). The results of this study demonstrate that the minimally invasive dorsal approach using the space between C-0 and C-1 or C-1 and C-2 provides direct and sufficient exposure for the safe surgical resection of small ventrally located intradural lesions at the

  7. Methylthioadenosine phosphorylase and activated insulin-like growth factor-1 receptor/insulin receptor: potential therapeutic targets in chordoma.

    Science.gov (United States)

    Sommer, Josh; Itani, Doha M; Homlar, Kelly C; Keedy, Vicki L; Halpern, Jennifer L; Holt, Ginger E; Schwartz, Herbert S; Coffin, Cheryl M; Kelley, Michael J; Cates, Justin M M

    2010-04-01

    Currently there is no effective chemotherapy for chordoma. Recent studies report co-expression of insulin-like growth factor-1 receptor (IGF1R) and its cognate ligand in chordoma, but it is unknown whether this receptor tyrosine kinase is activated in these tumours. Additionally, genetic studies have confirmed frequent deletions of chromosome 9p in chordomas, which encompasses the cyclin-dependent kinase inhibitor 2A (CDKN2A) locus. Another gene in this region, methylthioadenosine phosphorylase (MTAP), is an essential enzyme of the purine salvage pathway and has therapeutic relevance because MTAP-deficient cells are particularly sensitive to inhibitors of de novo purine synthesis. We investigated whether these pathways might be potential therapeutic targets for chordoma. Paraffin-embedded tissue samples from 30 chordomas were analysed by immunohistochemistry for expression of the phosphorylated isoforms of IGF1R or the insulin receptor (pIGF1R/pIR) and selected downstream signalling molecules, including BCL2-associated agonist of cell death protein (BAD). Expression of CDKN2A and MTAP proteins was also assessed. Skeletal chondrosarcomas, benign notochordal cell tumours, and fetal notochord were studied for comparison. Phosphorylated IGF1R/IR was detected in 41% of chordomas, together with activated downstream signalling molecules, and pIGF1R/pIR was absent in benign notochordal cell tumours and fetal notochord. Thirty-nine per cent of chordomas were negative for MTAP immunoreactivity. Patients with pIGF1R/pIR-positive tumours showed significantly decreased median disease-free survival in multivariate survival analysis (p = 0.036), whereas phosphorylation of BAD at serine-99 was found to be associated with a favourable prognosis (p = 0.002). Approximately 40% of chordomas demonstrate evidence of activation of the IGF1R/IR signalling pathway or loss of a key enzyme in the purine salvage pathway. Aberrant signalling cascades and disrupted metabolic pathways such as

  8. Multifocal metastatic chordoma to the soft tissues of the fingertips: a case report including sonographic features and a review of the literature.

    Science.gov (United States)

    Smith, Zachary; Girard, Nicole; Hansford, Barry G

    2018-03-01

    Chordoma is a rare, locally aggressive tumor which commonly metastasizes, most often to the lung, liver, and spine. In this case report, a 59-year-old male with history of sacral chordoma and pulmonary metastases presented to the emergency department with swelling and discoloration of multiple left fingertips. The initial radiographs led to a presumptive diagnosis of gout, which did not respond to medical therapy. An ultrasound demonstrated multiple solid masses with vascular hyperechoic septations which were subsequently biopsied and proven to be metastatic chordoma. Metastatic disease to the hand is a well documented but rare manifestation of many malignancies. The clinical presentation and radiographic features of multifocal hand metastases may mimic entities such as systemic deposition and granulomatous diseases. To the best of our knowledge, this is the first case report of soft tissue chordoma metastases to the fingertips as well as the first reported sonographic description of chordoma metastases.

  9. Intradural disc herniation: radiographic findings and surgical results with a literature review.

    Science.gov (United States)

    Kobayashi, Kazuyoshi; Imagama, Shiro; Matsubara, Yuji; Yoshihara, Hisatake; Hirano, Kenichi; Ito, Zenya; Ando, Kei; Ukai, Junichi; Muramoto, Akio; Shinjo, Ryuichi; Matsumoto, Tomohiro; Nakashima, Hiroaki; Ishiguro, Naoki

    2014-10-01

    To report a series of four cases of intradural disc herniation (IDH) with a review of the literature. IDH is a rare type of disc herniation. Preoperative diagnosis is difficult and IDH is only confirmed during surgery in most cases. Here, we describe four cases of IDH, including three with lumbar hernia and one with thoracic hernia. A retrospective chart review, surgical database query, and review of radiology reports are presented for each case, along with a literature review of IDH. Two of the four patients had a history of surgery at the same spinal level. Ring enhancement in gadolinium-enhanced MRI, an air image in computed tomography, and complete block in myelography were observed in the series. Surgery was performed with a transdural approach in all patients. One patient underwent transforaminal lumbar interbody fusion after postoperative recurrence. Three patients with lumbar involvement had nerve root symptoms preoperatively, but showed symptomatic improvement in the early postoperative period. In contrast, the patient with thoracic involvement had preoperative muscle weakness due to myelopathy symptoms, and had residual symptoms after surgery. IDH is a rare disease and characteristic imaging findings can be useful for diagnosis. Intraoperative findings lead to a definitive diagnosis in many cases and recognition of the pathological characteristics of IDH is important. Copyright © 2014 Elsevier B.V. All rights reserved.

  10. Comparison of Electrophysiological Outcomes of Tethered Cord Syndrome and Spinal Intradural Tumors: A Retrospective Clinical Study.

    Science.gov (United States)

    Pusat, Serhat; Kural, Cahit; Solmaz, Ilker; Temiz, Caglar; Kacar, Yunus; Tehli, Ozkan; Kutlay, Murat; Daneyemez, Mehmet; Izci, Yusuf

    2017-01-01

    Electrophysiological evaluation of the outcomes of spinal procedures is important for neurosurgeons. Somatosensorial evoked potentials (SSEPs) are used for electrophysiological evaluation of tethered cord syndrome (TCS) and spinal intradural tumors (SIT). The aim of this study was to document the electrophysiological outcomes of surgery for TCS and SIT and to compare the results based on the preoperative diagnosis. The data of 30 patients, who were operated for TCS and SIT between 2011 and 2013, were reviewed retrospectively. Surgical release of the spinal cord was performed for TCS and tumor removal was performed for SIT. Median and tibial nerve SSEPs at the left and right sides were measured at preoperative, early and late postoperative periods and compared statistically based on the diagnosis and the time of electrophysiological assessment. The diagnosis was TCS in 12 (40%) patients and SIT in 18 (60%) patients. There was a significant difference between preoperative, and early and late postoperative SSEPs values. Tibial nerve latencies were prolonged in the early postoperative, but shortened in the late postoperative period. In contrast, median nerve latencies were shortened in the early postoperative, but prolonged in the late postoperative period. There was no significant difference between the TCS and SIT groups based on the surgical intervention. Tibial nerve latency may be prolonged in the early postoperative period of TCS and SIT patients. However, electrophysiological changes were not predictive for these patients. Further studies with more patients are needed for other spinal lesions.

  11. Endoscope-assisted retrosigmoid intradural suprameatal approach for surgical treatment of trigeminal schwannomas.

    Science.gov (United States)

    Samii, Madjid; Alimohamadi, Maysam; Gerganov, Venelin

    2014-12-01

    Trigeminal schwannomas are the most common intracranial nonvestibular schwannomas, and the dumbbell-shaped subtype is the most challenging. To evaluate the efficiency and safety of the endoscope-assisted retrosigmoid intradural suprameatal approach (EA-RISA) for dumbbell trigeminal schwannomas and to compare EA-RISA with classic RISA. A retrospective study of all patients with trigeminal schwannomas was performed with a focus on dumbbell tumors. Tumors were classified according to a modified Samii classification. Extent of tumor removal, outcome, and morbidity rates in the 2 subgroups were compared. Twenty patients were enrolled: 8 had dumbbell-shaped tumors (type C1), 8 had middle fossa tumors (A1-3), 3 had extracranial extension (D2), and 1 had posterior fossa tumor. Gross total resection was achieved in 15 and near-total resection in 5 patients. In 4 patients with dumbbell tumors, the classic RISA (Samii approach) was used; EA-RISA was used in the other 4 patients. The extent of petrous apex drilling was determined individually on the basis of the anatomic variability of suprameatal tubercle and degree of tumor-induced petrous apex erosion; in 2 patients, only minimal drilling was needed. The endoscope was applied after microsurgical tumor removal and in 3 of 4 patients revealed a significant unrecognized tumor remnant in the anterolateral and superolateral aspects of the Meckel cave. Thus, the EA-RISA technique allowed gross total resection of the tumor. The EA-RISA enlarges the exposure obtained with the classic RISA. Its judicious use can help achieve safe and radical removal of dumbbell-shaped trigeminal schwannomas (C1 type).

  12. Intradural communication between dorsal rootlets of spinal nerves: their clinical significance.

    Science.gov (United States)

    Solmaz, Bilgehan; Tatarlı, Necati; Ceylan, Davut; Keleş, Evren; Çavdar, Safiye

    2015-06-01

    Anatomical and surgical textbooks give almost no attention to the intradural communications between dorsal rootlets of adjacent spinal nerves. These communications can be of significance in various neurosurgical procedures and clinical conditions of the region. The spinal cord of six formaldehyde-fixed cadavers was dissected from C1-S5. The dorsal rootlets of the spinal nerves were exposed via a posterior approach and communications between adjacent spinal nerves were documented. The frequency of communication between adjacent dorsal rootlets of the spinal nerves showed variations among spinal levels. Thirty-eight dorsal rootlet communications were observed in six cadavers (12 sides) and 20 (52.6%) were at cervical levels, 14 (36.8%) at thoracic levels, and four (10.5%) at lumbar levels. The majority of communications were observed on the left side (65.8%). Communications were most frequently observed at cervical (C4-C5, C5-C6) and upper thoracic (T1-T2) levels and seen least frequently at lower thoracic and lumbar levels. No communications were observed at sacral levels. Five types of communication were observed: I. oblique ascending, II. oblique descending III. short Y, IV. long Y and V shaped. None of the communication extended beyond one segment at any spinal level. The occurrence of such dorsal rootlet communications ranged from 3 to 7 for each cadaver and the mean was 4.8 ± 1.3. Histological sections from various levels of the dorsal rootlet communications showed that all consisted of myelinated fibers of varying diameters. Such communications may lead to misinterpretation of the pathology on the basis of clinical signs and symptoms and also should be considered in rhizotomy.

  13. Cervical Hemilaminoplasty with Miniplates in Long Segment Intradural Extramedullary Ependymoma: Case Report and Technical Note.

    Science.gov (United States)

    Oral, Sukru; Tumturk, Abdulfettah; Kucuk, Ahmet; Menku, Ahmet

    2018-01-01

    The surgical approaches for spinal tumors, to a great extent, have been developed in accordance with the developments in medical technology. Today, many surgical techniques are implemented as anterior, anterolateral, posterior, posterolateral and combined approaches. Due to its low morbidity, the posterior approach is the more preferred one. Laminectomy is a widely used technique, especially in neoplastic lesions. However, following laminectomy, there are numerous complications such as instability, kyphotic deformity and scar formation. In this paper, the excision of a tumor that was located intradural-extramedullary at the C3-C7 level with the cervical hemilaminoplasty technique is described. A 47-year-old female patient presented to our clinic with increasing complaints of neck and left arm pain, left arm numbness and searing pain for the last 10 years. On examination, hypoesthesia at the C4-7 dermatomes in the left upper extremity, an increase in deep tendon reflexes, and bilateral positive Hoffmann reflexes were observed. C3-C7 laminae were opened unilaterally on the right side with a midline skin incision. The laminae were drilled with a high-speed drill to provide a wide opening, both on the midline obliquely and from the border of the lamina-facet joint. After the tumor was totally excised, hemilaminae were placed into the previous position and reconstructed with mini-plates and screws. Cervical hemilaminoplasty provides a wide field of vision in tumor surgery of this region. Besides, the reconstruction of hemilaminae is important for stability. As the integrity of the spinal canal is preserved during reoperations of this region, the risk of complications is decreased.

  14. Endovascular reconstruction of unruptured intradural vertebral artery dissecting aneurysms with the Pipeline embolization device.

    Science.gov (United States)

    Kühn, Anna Luisa; Kan, Peter; Massari, Francesco; Lozano, J Diego; Hou, Samuel Y; Howk, Mary; Gounis, Matthew J; Wakhloo, Ajay K; Puri, Ajit S

    2016-10-01

    Dissecting aneurysms of the vertebral artery (VA) are difficult to treat using current surgical and endovascular techniques. To analyze retrospectively the efficacy and safety of flow diverters in the treatment of dissecting aneurysms of the vertebral artery. We identified six patients with six unruptured VA dissecting aneurysms either arising from the V4 or V3-V4 junction that were treated with the Pipeline embolization device (PED) at our institution between July 2012 and February 2015. Among other parameters, technical feasibility of the procedure, procedure-related complications, angiographic results, and clinical outcome were evaluated. PED placement was achieved in all cases and immediate angiography follow-up demonstrated intra-aneurysmal contrast stasis with parent artery preservation. A temporary episode of dysarthria was noted in one patient. Major procedure-related complications were not observed. The 6-month follow-up (n=6) demonstrated complete/near-complete aneurysm obliteration in five patients and partial obliteration in one. At the 1-year follow-up (n=5) stable complete aneurysm occlusion was seen in two patients. Two cases showed progression from near complete occlusion and partial occlusion at 6 months to complete occlusion and near complete occlusion. One cases showed unchanged near complete occlusion. No aneurysmal bleeding, in-stent stenosis or thromboembolic complication was seen. National Institutes of Health Stroke Scale and modified Rankin scale scores remained unchanged from admission to discharge. Our preliminary experience with the use of PED for the treatment of intradural VA dissecting aneurysms shows promising short-term results, making this technique a feasible and safe treatment option in patients suitable for this approach. However, long-term and larger cohort studies are needed to validate these results. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to

  15. Condrosarcoma vertebral: A propósito de un caso recidivado con invasión intradural

    OpenAIRE

    Navarrete Faubel, Francisco Enrique; Escribá Roca, Ismael

    2000-01-01

    Presentamos un caso de condosarcoma primario vertebral en un paciente de 17 años, que, tras el tratamiento quirúrgico, radio y quimioterapia adyuvante, sufrió una recidiva con invasión intradural a los 5 años de evolución. El paciente fue reintervenido realizándose una descompresión medular y liberación radicular, pero debido a la extensión tumoral, no hubo recuperación neurológica, quedando un síndrome medular incompleto con nivel T10. La poca frecuencia de este tumor en la columna, la dific...

  16. A small prospective study of chordomas treated with radiotherapy and razoxane

    Energy Technology Data Exchange (ETDEWEB)

    Rhomberg, W.; Boehler, F.K. [Dept. of Radiooncology, General Hospital, Feldkirch (Austria); Novak, H. [Dept. of Diagnostic Radiology, General Hospital, Feldkirch (Austria); Dertinger, S.; Breitfellner, G. [Dept. of Pathology, General Hospital, Feldkirch (Austria)

    2003-04-01

    Purpose: To evaluate the local effect of conventional photon irradiation in chordomas if the radiosensitizing agent razoxane is added. The rationale for this procedure were improved results previously seen in soft tissue and chondrosarcomas with this combination. Patients and Methods: Between 1988 and 1996, five patients with histologically confirmed chordomas of the skull base or the spine (three females, two males) were irradiated with 6- and 25-MeV photons under razoxane medication, one patient was treated with a telecobalt unit. Single doses of 180-200 cGy were given five times a week. The median total tumor dose was 63 Gy (range 54-67 Gy). Concomitantly, the radiosensitizer razoxane was administered at a dose of 125 mg twice daily p.o., median total dose 7.6 g. The drug was started 3-5 days before the first irradiation, and continued until the end of radiotherapy. Results: After a potential median follow-up time of 10 years, three of the five patients are alive and show neither symptoms nor signs of recurrence in CT or MR images. One patient with persistent sacral chordoma died after 8 years from cardiac insufficiency, and another patient died after 6.5 years from a bleeding complication following surgery for recurrence. The patients remained locally controlled for 5, 5.5+, 6.4, 11+, and 13+ years, respectively. Objective tumor regressions were noted in three of four patients with measurable disease. Acute side effects included mucosal reactions, two of five patients developed a leukopenia WHO grade 3 due to razoxane. Serious long-term complications were not observed. Conclusions: Although the patient series is small, there is an interesting trend in local control and survival. The cases are unselected, and the follow-up time is of considerable duration. The treatment can easily be performed at any institution and is tolerated fairly well. (orig.)

  17. Chordoma: clinical characteristics, management and prognosis of a case series of 25 patients

    International Nuclear Information System (INIS)

    Ferraresi, Virginia; Biagini, Roberto; Nuzzo, Carmen; Zoccali, Carmine; Marandino, Ferdinando; Vidiri, Antonello; Salducca, Nicola; Zeuli, Massimo; Giannarelli, Diana; Cognetti, Francesco

    2010-01-01

    Adequate surgery still remains the only curative treatment of chordoma. Interesting clinical data on advanced disease with molecularly targeted therapies were reported. We described the clinical outcome of a series of chordoma patients followed at Regina Elena National Cancer Centre of Rome from 2004 to 2008. Twenty-five consecutive patients with sacral (11 patients), spine (13 patients), and skull base (1 patient) chordoma went to our observation. Six patients (24%) had primary disease, 14(56%) a recurrent disease, and 5(20%) a metastatic spreading. Surgery was the primary option for treatment in 22 out of 25 patients. Surgical margins were wide in 5 (23%) and intralesional in 17(77%) patients; 3 out of 4 in-house treated patients obtained wide margins. After first surgery, radiotherapy (protons or high-energy photons) were delivered to 3 patients. One out of the 5 patients with wide margins is still without evidence of disease at 20 months from surgery; 2 patients died without evidence of disease after 3 and 36 months from surgery. Sixteen out of 17 (94%) patients with intralesional margins underwent local progression at a median time of 18 months with a 2-year local progression-free survival of 47%. The 5-year metastasis-free survival rate was 78.3%. Seventeen patients with locally advanced and/or metastatic disease expressing platelet-derived growth factor receptor (PDGFR) β were treated with imatinib mesylate. A RECIST stabilization of the disease was the best response observed in all treated cases. Pain relief with reduction in analgesics use was obtained in 6 out of 11 (54%) symptomatic patients. The 5- and 10-year survival rates of the entire series of patients were 76.7 and 59.7%, respectively. Despite progress of surgical techniques and the results obtained with targeted therapy, more effort is needed for better disease control. Specific experience of the multidisciplinar therapeutic team is, however, essential to succeed in improving patients

  18. Chordoma: clinical characteristics, management and prognosis of a case series of 25 patients.

    Science.gov (United States)

    Ferraresi, Virginia; Nuzzo, Carmen; Zoccali, Carmine; Marandino, Ferdinando; Vidiri, Antonello; Salducca, Nicola; Zeuli, Massimo; Giannarelli, Diana; Cognetti, Francesco; Biagini, Roberto

    2010-01-28

    Adequate surgery still remains the only curative treatment of chordoma. Interesting clinical data on advanced disease with molecularly targeted therapies were reported. We described the clinical outcome of a series of chordoma patients followed at Regina Elena National Cancer Centre of Rome from 2004 to 2008. Twenty-five consecutive patients with sacral (11 patients), spine (13 patients), and skull base (1 patient) chordoma went to our observation. Six patients (24%) had primary disease, 14(56%) a recurrent disease, and 5(20%) a metastatic spreading. Surgery was the primary option for treatment in 22 out of 25 patients. Surgical margins were wide in 5 (23%) and intralesional in 17(77%) patients; 3 out of 4 in-house treated patients obtained wide margins. After first surgery, radiotherapy (protons or high-energy photons) were delivered to 3 patients. One out of the 5 patients with wide margins is still without evidence of disease at 20 months from surgery; 2 patients died without evidence of disease after 3 and 36 months from surgery. Sixteen out of 17 (94%) patients with intralesional margins underwent local progression at a median time of 18 months with a 2-year local progression-free survival of 47%. The 5-year metastasis-free survival rate was 78.3%. Seventeen patients with locally advanced and/or metastatic disease expressing platelet-derived growth factor receptor (PDGFR) beta were treated with imatinib mesylate. A RECIST stabilization of the disease was the best response observed in all treated cases. Pain relief with reduction in analgesics use was obtained in 6 out of 11 (54%) symptomatic patients. The 5- and 10-year survival rates of the entire series of patients were 76.7 and 59.7%, respectively. Despite progress of surgical techniques and the results obtained with targeted therapy, more effort is needed for better disease control. Specific experience of the multi-disciplinary therapeutic team is, however, essential to succeed in improving patients

  19. Analysis of receptor tyrosine kinases (RTKs) and downstream pathways in chordomas.

    Science.gov (United States)

    Tamborini, Elena; Virdis, Emanuela; Negri, Tiziana; Orsenigo, Marta; Brich, Silvia; Conca, Elena; Gronchi, Alessandro; Stacchiotti, Silvia; Manenti, Giacomo; Casali, Paolo G; Pierotti, Marco A; Pilotti, Silvana

    2010-08-01

    We have previously demonstrated that chordomas express activated platelet-derived growth factor receptor (PDGFRB) and that treatment with imatinib, which is capable of switching off the activation of various receptor tyrosine kinases (RTKs) including PDGFRB, benefits a number of patients. The aim of this study was to identify the possible presence of other activated RTKs and their downstream signaling effectors. Cryopreserved material from 22 naïve sporadic chordomas was investigated for the presence of activated RTKs and their cognate ligands and downstream signaling effectors by means of human phospho-RTK antibody arrays, Western blotting, and molecular analysis; immunohistochemistry and fluorescence in situ hybridization were used to analyze the corresponding formalin-fixed and paraffin-embedded samples. We detected activated PDGFRB, FLT3, and colony stimulating factor 1 receptor (CSF1R) of the PDGFR family and highly phosphorylated EGFR, HER2/neu, and (to a lesser extent) HER4 of the EGFR family. The detection of PDGFRB/PDGFB confirmed our previous data. The presence of activated EGFR was paralleled by the finding of high levels of epidermal growth factor (EGF) and transforming growth factor alpha (TGFalpha) and PDGFB co-expression and PDGFRB co-immunoprecipitation. Of the downstream effectors, the PI3K/AKT and RAS/MAPK pathways were both activated, thus leading to the phosphorylation of mammalian target of rapamycin (mTOR) and 4E-BP1 among the regulators involved in translational control. Taken together, our results (i) provide a rationale for tailored treatments targeting upstream activated receptors, including the PDGFR and EGFR families; (ii) support the idea that a combination of upstream antagonists and mTOR inhibitors enhances the control of tumor growth; and (iii) indicate that the 4E-BP1/eIF4E pathway is a major regulator of protein synthesis in chordoma.

  20. Case report 357: Chordoma of the fourth lumbar vertebra metastasizing to the thoracic spine and ribs

    International Nuclear Information System (INIS)

    Abdelwahab, I.F.; Zwass, A.; O'Leary, P.F.; Lenox Hill Hospital, New York, NY; Beth Israel Medical Center, New York, NY; Steiner, G.C.

    1986-01-01

    In summary a fascinating case is presented in a 54-year-old man who developed a chordoma of the fourth lumbar vertebra which was treated by radiotherapy, with good results. The man remained asymptomatic relatively for several years and then presented with recurrence of back pain and neurological deficits. Plain films, CT and myelography showed considerable destruction of the body of L4 with a sclerotic pattern suggesting the effects of previous radiotherapy. A large paraspinal tissue mass extending into the spinal canal was present. Most interestingly the patient developed metastatic disease in the thoracic spine and ribs but no metastases other than in the skeleton. (orig./SHA)

  1. The analysis of the effective of preserving sacral nerve root during surgical treatment of chordoma

    International Nuclear Information System (INIS)

    Ji Yiming; Chen Kangwu; Yang Huilin; Zhu Lifan

    2010-01-01

    Objective: To analyze the effective of preserving sacral nerve root during surgical treatment of sacral chordoma. Methods: This retrospective study included 30 cases of sacral chordomas. All the cases were operated with posterior approach. The blood loss and blood transfusion during operation, the drainged blood after operation were reviewed. The sphincter muscle function of bladder and bowl were observed. Results: Tremendous reduction of blood loss during surgery was found in all cases, the blood loss was 1280 ml in average, the blood transfusion was 1080 ml in average, the drainged blood after ope-ration was 650 ml. Nine patients whose sacral nerve roots had been reserved bilaterally at and above S 3 level, the sphincter muscle function of bladder and bowl was good, whereas the function of sphincter muscle impaired in the other 21 patients and in one case colostomy and ureterocutaneostomy were used. Conclusion: Preoperative arterial embolization is effective method and can lead to excellent results. Even if the tumor is relatively huge and the upper resection margin is as high as at S 1 or S 2 level, the tumor can be removed successfully by posterior approach. Sacral nerve should be preserved as possible. (authors)

  2. A Case of Laterally Extended High-Positioned Chordoma Treated Using the High Cervical Retropharyngeal Approach.

    Science.gov (United States)

    Ito, Kiyoshi; Nakamura, Takuya; Aoyama, Tatsuro; Horiuchi, Tetsuyoshi; Hongo, Kazuhiro

    2017-09-01

    Several surgical approaches for the treatment of pathologies of the craniovertebral junction (CVJ) and high cervical regions have been reported. For the best postoperative results, selection of a surgical route to treat such pathologies should be based on a complete understanding of the approach. A 64-year-old woman presented with a 5-year history of motor and sensory disturbances in her right upper extremity. Cervical magnetic resonance imaging (MRI) showed a slightly enhanced mass at the C2-C4 level. Sagittal T2-weighted MRI revealed a hyperintense dumbbell-shaped mass involving a damaged C3 vertebral body. We performed a 2-stage operation to achieve gross total removal of the tumor. In the first operation, a posterior approach was used to remove the intracanalicular tumor, achieve spinal cord decompression, and establish a histological diagnosis of the tumor (subsequently diagnosed as a chordoma). In the second operation, gross total removal of the chordoma was achieved via the anterior high cervical retropharyngeal approach. We used iliac bone and titanium plates for the bony fusion. Our results indicate that the high cervical retropharyngeal approach is a reasonable option for pathologies located in the anterior or anterolateral portions of high cervical regions. This approach is an alternative to the transoral approach to the ventral CVJ and high cervical regions. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Extended maxillotomy for skull base access in contemporary management of chordomas: Rationale and technical aspect.

    Science.gov (United States)

    Abdul Jalil, Muhammad Fahmi; Story, Rowan D; Rogers, Myron

    2017-05-01

    Minimally invasive approaches to the central skull base have been popularized over the last decade and have to a large extent displaced 'open' procedures. However, traditional skull base surgery still has its role especially when dealing with a large clival chordoma where maximal surgical resection is the principal goal to maximize patient survival. In this paper, we present a case of a 25year-old male patient with chordoma in the inferior clivus which was initially debulked via a transnasal endoscopic approach. He unfortunately had a large recurrence of tumor requiring re-do resection. With the aim to achieve maximal surgical resection, we then chose the technique of a transoral approach with Le Fort 1 maxillotomy and midline palatal split. Post-operative course for the patient was uneventful and post-operative MRI confirmed significant debulking of the clival lesion. The technique employed for the surgical procedure is presented here in detail as is our experience over two decades using this technique for tumors, inflammatory lesions and congenital abnormalities at the cranio-cervical junction. Copyright © 2017 Elsevier Ltd. All rights reserved.

  4. Auto-immune thyroid dysfunction induced by tyrosine kinase inhibitors in a patient with recurrent chordoma.

    Science.gov (United States)

    Eroukhmanoff, Juliette; Castinetti, Frederic; Penel, Nicolas; Salas, Sebastien

    2016-08-24

    While hypothyroidism has frequently been reported with the use of TKIs, the thyroid-stimulating hormone (TSH) suppressing effect of TKIs is rare, except for thyroiditis. We describe a case with progressive recurrent chordoma who initially became hyperthyroid in a context of autoimmunity under sorafenib treatment and later under imatinib treatment. A 57-year-old man with lumbar chordoma began daily treatment of 800 mg sorafenib. He did not have any other medication or recent iodinated-contrast exposure and his family history was negative for thyroid and autoimmune disease. There was no history of neck pain, irradiation or trauma, recent fever or viral illness. Pre-treatment TSH was normal. After 18 weeks of treatment, the patient presented hyperthyroidism with positive anti-TSH receptor antibodies. More surprisingly, Graves' disease recurred during treatment with imatinib. The fact that Graves' disease occurred after two different TKIs suggests that it could be a rare but important class effect. Anti-TSH receptor antibodies should be systematically measured when TSH decreases in order to avoid the erroneous diagnosis of transient hyperthyroidism due to thyroiditis.

  5. Lymphocyte-Related Inflammation and Immune-Based Scores Predict Prognosis of Chordoma Patients After Radical Resection

    Directory of Open Access Journals (Sweden)

    Wenhao Hu

    2018-04-01

    Full Text Available The inflammatory microenvironment plays a critical role in the development and progression of malignancies. In the present study, we aimed to evaluate the prognostic value of lymphocyte-related inflammation and immune-based prognostic scores in patients with chordoma after radical resection, including the neutrophil-lymphocyte ratio (NLR, platelet-lymphocyte ratio (PLR, monocyte-lymphocyte ratio (MLR, and systemic immune-inflammation index (SII. A total of 172 consecutive patients with chordoma who underwent radical resection were reviewed. R software was used to randomly select 86 chordoma patients as a training set and 86 chordoma patients as a validation set. Potential prognostic factors were also identified, including age, sex, tumor localization, KPS, Enneking stage, tumor size, and tumor metastasis. Overall survival (OS was calculated using the Kaplan–Meier method and multivariate Cox regression analyses. NLR, PLR, SII, Enneking stage, tumor differentiation and tumor metastasis were identified as significant factors from the univariate analysis in both the training and validation sets and were subjected to multivariate Cox proportional hazards analysis. The univariate analysis showed that NLR ≥1.65, PLR ≥121, and SII ≥370×109/L were significantly associated with poor OS. In the multivariate Cox proportional hazard analysis, SII, Enneking stage and tumor metastasis were significantly associated with OS. As noninvasive, low-cost, reproducible prognostic biomarkers, NLR, PLR and SII could help predict poor prognosis in patients with chordoma after radical resection. This finding may contribute to the development of more effective tailored therapy according to the characteristics of individual tumors.

  6. Chordoma of the petrous apex - a case report and review of the literature; Cordoma de apice petroso - relato de um caso e revisao da literatura

    Energy Technology Data Exchange (ETDEWEB)

    Loureiro, Ricardo; Leal Junior, Osvaldo S. [Pernambuco Univ., Recife, PE (Brazil). Faculdade de Medicina; Loureiro, Lautonio Junior [Universidade de Pernambuco, Recife, PE (Brazil). Faculdade de Ciencias Medicas; Buril, Marlus V.M. [Hospital das Clinicas, Recife, PE (Brazil). Centro de Terapia Intensiva

    1998-10-01

    Chordomas are rare tumours arising from remnants of the embryologic notochord, typically at a midline position. Although 35-40% of these lesions are intracranial, these tumors answer for less than 1% of all intracranial tumors. The intracranial chordomas originate most frequently from the clival region at the midline. Nevertheless eventually may arise off the midline primarily in petrous apex or, very rarely, in paranasal sinuses. The authors report a case of histopathologically proved intracranial chordoma that arose atypical site in the petrous apex. The computed tomographic and magnetic resonance imaging finding were similar to those observed in midline chordomas. The computed tomographic examination revealed a well-defined soft tissue mass associated with bone destruction and foci of calcification. The magnetic resonance imaging study demonstrated a growing extra-axial formation that appeared with hypo-intensity of signal on T1-weighted images, hyperintensity on T2-weighted images and heterogeneous enhancement after paramagnetic agent injection. (author) 8 refs., 8 figs.

  7. Brachyury, SOX-9, and Podoplanin, New Markers in the Skull Base Chordoma Vs Chondrosarcoma Differential: A Tissue Microarray Based Comparative Analysis

    Science.gov (United States)

    Oakley, GJ; Fuhrer, K; Seethala, RR

    2014-01-01

    The distinction between chondrosarcoma and chordoma of the skull base/head and neck is prognostically important; however, both have sufficient morphologic overlap to make distinction difficult. As a result of gene expression studies, additional candidate markers have been proposed to help in this distinction. Hence, we sought to evaluate the performance of new markers: brachyury, SOX-9, and podoplanin alongside the more traditional markers glial fibrillary acid protein, carcinoembryonic antigen, CD24 and epithelial membrane antigen. Paraffin blocks from 103 skull base/head and neck chondroid tumors from 70 patients were retrieved (1969-2007). Diagnoses were made based on morphology and/or whole section immunohistochemistry for cytokeratin and S100 protein yielding 79 chordomas (comprising 45 chondroid chordomas and 34 conventional chordomas), and 24 chondrosarcomas. A tissue microarray containing 0.6 mm cores of each tumor in triplicate was constructed using a manual array (MTA-1, Beecher Instruments). For visualization of staining, the ImmPRESS detection system (Vector Laboratories) with 2 - diaminobenzidine substrate was used. Sensitivities and specificities were calculated for each marker. Core loss from the microarray ranged from 25-29% yielding 66-78 viable cases per stain. The classic marker, cytokeratin, still has the best performance characteristics. When combined with brachyury, accuracy improves slightly (sensitivity and specificity for detection of chordoma 98% and 100%, respectively). Positivity for both epithelial membrane antigen and AE1/AE3 had a sensitivity of 90% and a specificity of 100% for detecting chordoma in this study. SOX-9 is apparently common to both notochordal and cartilaginous differentiation, and is not useful in the chordoma-chondrosarcoma differential diagnosis. Glial fibrillary acid protein, carcinoembryonic antigen, CD24, and epithelial membrane antigen did not outperform other markers, and are less useful in the diagnosis of

  8. A Prospective Outcomes Study of Proton Therapy for Chordomas and Chondrosarcomas of the Spine

    Energy Technology Data Exchange (ETDEWEB)

    Indelicato, Daniel J., E-mail: dindelicato@floridaproton.org [Department of Radiation Oncology, University of Florida College of Medicine, Jacksonville, Florida (United States); Rotondo, Ronny L.; Begosh-Mayne, Dustin [Department of Radiation Oncology, University of Florida College of Medicine, Jacksonville, Florida (United States); Scarborough, Mark T.; Gibbs, C. Parker [Department of Orthopedics and Rehabilitation, University of Florida College of Medicine, Gainesville, Florida (United States); Morris, Christopher G.; Mendenhall, William M. [Department of Radiation Oncology, University of Florida College of Medicine, Jacksonville, Florida (United States)

    2016-05-01

    Purpose: To evaluate the effectiveness of definitive or adjuvant external beam proton therapy on survival in patients with chordomas and chondrosarcomas of the spine. Methods and Materials: Between March 2007 and May 2013, 51 patients with a median age of 58 years (range, 22-83 years) with chordoma (n=34) or chondrosarcomas (n=17) of the sacrum (n=21), the cervical spine (n=20), and the thoracolumbar spine (n=10) were treated with external beam proton therapy to a median dose of 70.2 Gy(RBE) [range, 64.2-75.6 Gy(RBE)] at our institution. Distant metastases, overall survival, cause-specific survival, local control, and disease-free survival were calculated. Results: The mean follow-up time was 3.7 years (range, 0.3-7.7 years). Across all time points, 25 patients experienced disease recurrence: 18 local recurrences, 6 local and distant recurrences, and 1 distant metastasis. The 4-year rates of overall survival and cause-specific survival were 72%; disease-free survival was 57%, local control was 58%, and freedom from distant metastases was 86%. The median time to local progression was 1.7 years (range, 0.2-6.0 years), and the median time to distant progression was 1.6 years (range, 0.2-6.0 years). The risk factors for local recurrence were age ≤58 years (62% vs 26%; P=.04) and recurrence after prior surgery (29% vs 81%; P=.01). Secondary cancers developed in 2 patients: B-cell lymphoma 5.5 years after treatment and bladder cancer 2 years after treatment. We observed the following toxicities: sacral soft tissue necrosis requiring surgery (n=2), T1 vertebral fracture requiring fusion surgery (n=1), chronic urinary tract infections (n=1), surgery for necrotic bone cyst (n=1), and grade 2 bilateral radiation nephritis (n=1). Conclusion: High-dose proton therapy controls more than half of spinal chordomas and chondrosarcomas and compares favorably with historic photon data. Local progression is the dominant mode of treatment failure and may be reduced by

  9. Influence of Residual Tumor Volume and Radiation Dose Coverage in Outcomes for Clival Chordoma

    Energy Technology Data Exchange (ETDEWEB)

    McDonald, Mark W., E-mail: markmcdonaldmd@gmail.com [Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana (United States); Indiana University Health Proton Therapy Center, Bloomington, Indiana (United States); Linton, Okechukwu R. [Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana (United States); Moore, Michael G.; Ting, Jonathan Y. [Department of Otolaryngology, Head and Neck Surgery, Indiana University School of Medicine, Indianapolis, Indiana (United States); Cohen-Gadol, Aaron A.; Shah, Mitesh V. [Department of Neurological Surgery, Indiana University School of Medicine, Indianapolis, Indiana (United States); Goodman Campbell Brain and Spine, Indianapolis, Indiana (United States)

    2016-05-01

    Purpose: The purpose of this study was to evaluate factors associated with tumor control in clival chordomas. Methods and Materials: A retrospective review of 39 patients treated with surgery and proton therapy for clival chordomas between 2004 and 2014 was performed. The median prescribed dose was 77.4 Gy (relative biological effectiveness [RBE]); range was 70.2-79.2 Gy (RBE). Minimum and median doses to gross tumor volume (GTV), radiation dose received by 1 cm{sup 3} of GTV (D1cm{sup 3}), and the equivalent uniform dose were calculated. Receiver operating characteristics curves evaluated the predictive sensitivity and specificity for local failure of potential cutpoint values for GTV and D1cm{sup 3}. Results: After a median follow-up of 51 months, the 5-year estimate of local control (LC) was 69.6% (95% confidence interval [CI] 50.0%-89.2%), and overall survival (OS) was 81.4% (95% CI: 65.3%-97.5%). Tumor histology, GTV at the time of radiation, and prescribed radiation dose were significantly associated with local control on multivariate analysis, whereas D1cm{sup 3} was associated with overall survival. Compared to those patients whose conditions remained controlled, patients experiencing tumor failure had statistically significant larger GTVs and lower D1cm{sup 3}, and prescribed and median doses to GTV. A subset of 21 patients with GTV of ≤20 cm{sup 3} and D1cm{sup 3} of >67 Gy (RBE) had a median follow-up of 47 months. The 5-year estimate of local control in this subset was 81.1% (95% CI: 61.7%-100%; P=.004, overall comparison by GTV ≤20 cm{sup 3} stratified by D1cm{sup 3}). A D1cm{sup 3} of 74.5 Gy (RBE) had 80% sensitivity for local control and 60% specificity, whereas a GTV of 9.3 cm{sup 3} had 80% sensitivity for local control and 66.7% specificity. Conclusions: Local control of clival chordomas was associated with both smaller size of residual tumor and more complete high-dose coverage of residual tumor. Multidisciplinary care should seek

  10. Chordoma: clinical characteristics, management and prognosis of a case series of 25 patients

    Directory of Open Access Journals (Sweden)

    Giannarelli Diana

    2010-01-01

    Full Text Available Abstract Background Adequate surgery still remains the only curative treatment of chordoma. Interesting clinical data on advanced disease with molecularly targeted therapies were reported. Methods We described the clinical outcome of a series of chordoma patients followed at Regina Elena National Cancer Centre of Rome from 2004 to 2008. Results Twenty-five consecutive patients with sacral (11 patients, spine (13 patients, and skull base (1 patient chordoma went to our observation. Six patients (24% had primary disease, 14(56% a recurrent disease, and 5(20% a metastatic spreading. Surgery was the primary option for treatment in 22 out of 25 patients. Surgical margins were wide in 5 (23% and intralesional in 17(77% patients; 3 out of 4 in-house treated patients obtained wide margins. After first surgery, radiotherapy (protons or high-energy photons were delivered to 3 patients. One out of the 5 patients with wide margins is still without evidence of disease at 20 months from surgery; 2 patients died without evidence of disease after 3 and 36 months from surgery. Sixteen out of 17 (94% patients with intralesional margins underwent local progression at a median time of 18 months with a 2-year local progression-free survival of 47%. The 5-year metastasis-free survival rate was 78.3%. Seventeen patients with locally advanced and/or metastatic disease expressing platelet-derived growth factor receptor (PDGFR β were treated with imatinib mesylate. A RECIST stabilization of the disease was the best response observed in all treated cases. Pain relief with reduction in analgesics use was obtained in 6 out of 11 (54% symptomatic patients. The 5- and 10-year survival rates of the entire series of patients were 76.7 and 59.7%, respectively. Conclusions Despite progress of surgical techniques and the results obtained with targeted therapy, more effort is needed for better disease control. Specific experience of the multidisciplinar therapeutic team is

  11. Chordoma of the thoracic vertebrae in a Bengal tiger (Panthera tigris tigris).

    Science.gov (United States)

    Kuramochi, Mizuki; Izawa, Takeshi; Hori, Mayuka; Kusuda, Kayo; Shimizu, Junichiro; Iseri, Toshie; Akiyoshi, Hideo; Ohashi, Fumihito; Kuwamura, Mitsuru; Yamate, Jyoji

    2015-07-01

    A 19-year-old female Bengal tiger (Panthera tigris tigris) was presented with hind limb weakness, ataxia and respiratory distress. Computed tomography revealed a mass between the left side of the T7 vertebra and the base of the left 7th rib. The tiger then died, and necropsy was performed. Grossly, the vertebral mass was 6 × 5.7 × 3 cm, and invaded the adjacent vertebral bone and compressed the T7 spinal cord. Histologically, the mass was composed of large, clear, vacuolated and polygonal cells with osteochondral matrix. Cellular and nuclear atypia were moderate. The vacuolated cells stained positively for cytokeratin and vimentin and negatively for S-100. Based on these findings, the present case was diagnosed as a vertebral chordoma; the first report in a tiger.

  12. IMAGING DIAGNOSIS-MAGNETIC RESONANCE IMAGING AND HISTOPATHOLOGICAL FEATURES OF A SKULL BASE CHORDOMA IN A CAT.

    Science.gov (United States)

    Brocal, Josep; Gamino, Virginia; Guevar, Julien; Gutierrez-Quintana, Rodrigo; Marchesi, Francesco; Hammond, Gawain; Stalin, Catherine

    2017-03-01

    An 8-year-old domestic short-haired cat was presented with anorexia, lethargy, ataxia and one episode of consciousness loss. A midline vertically orientated, biconcave, extra-axial mass originating from the basioccipital bone was detected on magnetic resonance images of the head. The mass was T1W iso- to hypointense when compared with normal grey matter, T2W hyperintense with small areas of isointensity and heterogeneously enhanced with contrast. Multiple signal voids were observed on T2 * images. Histopathological evaluation confirmed a chordoma. To the authors' knowledge this is the first report of the imaging characteristics of a chordoma affecting the skull base in a cat. © 2016 American College of Veterinary Radiology.

  13. Combined use of maxillomandibular swing approach and neurosurgical ultrasonic aspirator in the management of extensive clival chordoma: A case report

    Directory of Open Access Journals (Sweden)

    Hassan Shahid

    2008-02-01

    Full Text Available Abstract Introduction Chordoma is a rare malignant tumour with an incidence of metastasis of less than 10 percent. Usually arising from clivus its posterior extension may involve the brainstem before presenting as nasal mass and obstruction. Surgery is the main mode of treatment with adjuvant radiotherapy. However surgery is rarely possible for a large intracranial lesion. Case presentation We report the case of an adolescent patient with a chordoma extending posteriorly to the brainstem and anteriorly to the nasopharynx and managed by the combination of resection using a maxillomandibular swing approach and the use of a neurosurgical ultrasonic aspirator. Conclusion Maxillomandibular swing approach provides good access for large nasopharyngeal tumour extending brainstem area.

  14. Giant notochordal hamartoma of intraosseous origin: a newly reported benign entity to be distinguished from chordoma. Report of two cases

    International Nuclear Information System (INIS)

    Mirra, J.M.; Brien, E.W.

    2001-01-01

    Two cases are reported of a newly described intraosseous entity of vertebral bodies deemed ''giant notochordal hamartoma of intraosseous origin''. This entity is commonly mistaken for chordoma and must be distinguished from it as the consequences of misinterpretation may be serious. The clinical, radiological and histologic criteria that can be used to distinguish these two entities are emphasized. Included is a proposed pathogenesis for this lesion, its probable notochordal origin, and a review of other probable cases. (orig.)

  15. EGFR Inhibition in a Pretreated Sacral Chordoma: A Role for Erlotinib? Case Report and a Brief Review of Literature.

    Science.gov (United States)

    Trapani, D; Conforti, F; De Pas, T

    2017-01-01

    We describe the case of a 69-year old male with an EGFR- positive Imatinib refractory sacral chordoma with synchronous lung metastases, treated with erlotinib, a first-generation EGFR inhibitor. After disease progression following first-line Imatinib and a combination therapy with everolimus plus metformin, we made a challenge with an EGFR tyrosine kinase inhibitor (EGFR TKI), erlotinib. Despite a brief clinical benefit, the patient presented a rapid clinical deterioration leading to death, after 8 weeks of treatment.

  16. EGFR Inhibition in a Pretreated Sacral Chordoma: A Role for Erlotinib? Case Report and a Brief Review of Literature

    OpenAIRE

    Trapani, D.; Conforti, F.; De Pas, T.

    2017-01-01

    We describe the case of a 69-year old male with an EGFR- positive Imatinib refractory sacral chordoma with synchronous lung metastases, treated with erlotinib, a first-generation EGFR inhibitor. After disease progression following first-line Imatinib and a combination therapy with everolimus plus metformin, we made a challenge with an EGFR tyrosine kinase inhibitor (EGFR TKI), erlotinib. Despite a brief clinical benefit, the patient presented a rapid clinical deterioration leading to death, a...

  17. Intradural spine surgery may not carry an increased risk of shunt revision compared with extradural spine surgery in pediatric patients with myelomeningocele.

    Science.gov (United States)

    Kuhn, Elizabeth N; Hopson, Betsy; Conklin, Michael J; Blount, Jeffrey P

    2018-01-01

    OBJECTIVE Patients with myelomeningocele are often affected by scoliosis and tethered cord syndrome, and frequently require spine surgery. Intradural spine surgeries may carry an inherently higher risk of inducing shunt malfunction due to entry into the subarachnoid space. In this study, the authors sought to compare rates of shunt malfunction after intradural and extradural spine surgeries among pediatric patients with myelomeningocele. METHODS The authors reviewed records of the National Spina Bifida Program Registry for Children's Hospital of Alabama. The Exago reporting function was used to identify patients who had received at least one of the following procedures: shunt revision, tethered cord release (TCR), or spinal fusion for deformity. The registry records were reviewed for all identified patients to determine if a shunt revision was performed within the 1st year after TCR or spinal fusion. RESULTS Final analyses included 117 patients, of whom 39 underwent spinal fusion and 78 underwent TCR. Among patients who underwent spinal fusion, shunt revision was performed within 30 days in 2 patients (5.1%), within 60 days in 2 (5.1%), within 90 days in 4 (10.3%), and within 1 year in 5 (12.8%). Among patients who underwent TCR, shunt revision was performed within 30 days in 7 patients (9.0%), within 60 days in 10 (12.8%), within 90 days in 11 (14.1%), and within 1 year in 17 (21.8%). Using the log-rank test, there was no significant difference in Kaplan-Meier curves between intradural and extradural groups (p = 0.59). CONCLUSIONS In a review of single-institution registry data, the authors found no statistically significant difference in the risk of shunt malfunction after intradural and extradural spine surgeries.

  18. Spontaneous cervical intradural disc herniation presenting with Brown-Séquard and Horner's syndrome: lesson learned from a very unique case.

    Science.gov (United States)

    Baudracco, Irene; Grahovac, Gordan; Russo, Vittorio M

    2017-05-01

    Cervical spontaneous intradural disc herniation (IDH) is an extremely rare condition. We describe a unique case of a patient presenting with a Brown-Séquard syndrome (BSS) and Horner's syndrome (HS). This study aimed to report an unusual case of spontaneous cervical intradural disc herniation that presented with Horner's and Brown-Séquard syndrome (BSS) and discuss difficulties in preoperative diagnosis and treatment difficulties of intradural cervical disc. Notes and images review, and analysis of the relevant literature. A 45-year old female presented with acute Horner's syndrome and Brown-Séquard syndrome. The magnetic resonance imaging of cervical spine revealed C4-5 disc extrusion with cord compression. The patient underwent urgent decompression through an anterior cervical corpectomy and fusion. Patient fully recovered 6 months after disease onset. We would like to emphasize that prompt and anterior cervical decompression is the treatment of choice, as it directly address the problem and allows dura repair in spontaneous cervical disc herniation.

  19. Spinal perimedullary vein enlargement sign: an added value for the differentiation between intradural-extramedullary and intramedullary tumors on magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Gong, Tao; Wang, Guangbin; Gao, Fei; Chen, Xin [Shandong University, Department of Shandong Medical Imaging Research Institute, Jinan (China); Liu, Yubo [Provincial Hospital Affiliated to Shandong University, Department of Radiology, Jinan (China); Yang, Li [Zhongshan Hospital, Department of Radiology, Shanghai (China); Chen, Weibo [Philips Healthcare, Shanghai (China)

    2016-11-15

    The purpose of this study was to determine the added value of the perimedullary spinal vein enlargement sign on magnetic resonance imaging (MRI) in distinguishing intradural-extramedullary tumors (IDEMTs) from intramedullary spinal tumors (IMTs). Two hundred and eight consecutive spinal intradural tumors with histopathologic confirmation (21 IMTs, 187 IDEMTs) were enrolled. Two readers blinded to the final pathological diagnosis and clinical data independently assessed the venous enlargement sign to determine the agreement between them and jointly distinguished IDEMTs from IMTs according to the common MRI findings. Sensitivity, specificity, and accuracy for the diagnosis of IDEMTs were calculated for the common MRI findings, vein enlargement sign, and a combination of both. Intraobserver agreement and interobserver agreement for both readers was excellent. The sensitivity, specificity, and accuracy of common MRI findings for differentiating IDEMTs from IMTs were 83.4, 95.2, and 89.3 %, respectively. Thirty-one IDEMTs were mistakenly diagnosed as IMTs, in which seven were cases with vein enlargement signs. By applying the vein enlargement sign to the common MRI findings, the specificity remained at 95.2 %, while the sensitivity improved to 89.3 % and the accuracy increased to 92.3 %. The spinal perimedullary vein enlargement sign is useful in assessing intradural tumors and to differentiate IDEMTs from IMTs. (orig.)

  20. Whole-Body MRI Virtual Autopsy Using Diffusion-weighted Imaging With Background Suppression (DWIBS) at 3 T in a Child Succumbing to Chordoma.

    Science.gov (United States)

    Andronikou, Savvas; Kemp, Marnie L; Meiring, Michelle

    2017-03-01

    We report the use of diffusion-weighted imaging with background suppression (DWIBS) in pediatric virtual magnetic resonance imaging (MRI) autopsy of a child who succumbed to chordoma. A 10-year-old girl who succumbed to relapse of a chordoma underwent whole-body virtual MRI autopsy 12 hours postmortem with short Tau inversion recovery (STIR) and DWIBS on 3 T, which demonstrated the primary mass, local and cardiac invasion, and metastatic disease to the thorax, abdomen, head/neck, and musculoskeletal system. Postmortem virtual MRI autopsy including DWIBS successfully demonstrated the transthoracic spread of chordoma and invasion of the heart, resulting in blood-borne metastases. Motion and respiratory artifact were not factors during virtual autopsy using DWIBS on 3 T, making ideal use of this technology.

  1. Quantitative comparison of retrosigmoid intradural suprameatal approach and retrosigmoid transtentorial approach: implications for tumors in the petroclival region.

    Science.gov (United States)

    Ambekar, Sudheer; Amene, Chiazo; Sonig, Ashish; Guthikonda, Bharat; Nanda, Anil

    2013-10-01

    Background Retrosigmoid transtentorial (RTT) and retrosigmoid intradural suprameatal (RISA) approaches have been used in the treatment of petroclival tumors. Objective To compare the area of exposure of brainstem and petroclival region obtained through RTT and RISA in cadaveric specimens. Methods Five cadaveric specimens with a total of 10 sides were analyzed. RTT and RISA were performed on five sides each. Brainstem and petroclival surface exposure were measured using both the approaches. These values were compared between the two approaches. Results Brainstem area exposure with RTT was 441 ± 63 mm(2) and that with RISA was 311 ± 61 mm(2). Student's t-test revealed that the difference was significant (p = 0.01). The area of petroclival exposure medial to the Meckel cave through RTT was 696 ± 57 mm(2), and that through RISA was 716 ± 51 mm(2) (p = 0.69). The area of brainstem exposure between V and VII-VII complex through RTT and RISA was 387 ± 86 mm(2) and 378 ± 76 mm(2) (p = 0.87). Conclusion The RTT approach is an excellent approach to ventrolateral brainstem and petroclival region. It provides greater superoventral exposure of the ventrolateral brainstem than RISA and provides similar petroclival exposure.

  2. Flow-diverting stents allow efficient treatment of unruptured, intradural dissecting aneurysms of the vertebral artery: An explanatory approach using in vivo flow analysis.

    Science.gov (United States)

    Gölitz, Philipp; Struffert, Tobias; Hoelter, Philip; Eyüpoglu, Ilker; Knossalla, Frauke; Doerfler, Arnd

    2016-02-01

    Our study aimed to evaluate the efficiency of flow-diverting stents (FDS) in treating unruptured, intradural dissecting aneurysms of the vertebral artery (VADAs). Additionally, the effect of FDS on the aneurysmal flow pattern was investigated by performing in vivo flow analysis using parametric color coding (PCC). We evaluated 11 patients with unruptured, intradural VADAs, treated with FDS. Pre- and postinterventional DSA-series were postprocessed by PCC, and time-density curves were calculated. The parameters aneurysmal inflow-velocity, outflow-velocity and relative time-to-peak (rTTP) were calculated. Pre- and postinterventional values were compared and correlated with the occlusion rate after six months. Follow-up DSA detected 10 aneurysms occluded, meaning an occlusion rate of 91%. No procedure-related morbidity and mortality was found. Flow analyses revealed a significant reduction of aneurysmal inflow- velocity and prolongation of rTTP after FDS deployment. Concerning aneurysm occlusion, the postinterventional outflow-velocity turned out to be a marginally statistically significant predictor. A definite threshold value (-0.7 density change/s) could be determined for the outflow-velocity that allows prediction of complete aneurysm occlusion with high sensitivity and specificity (100%). Using FDS can be considered an efficient and safe therapy option in treating unruptured, intradural VADA. From in vivo flow analyses the postinterventional aneurysmal outflow-velocity turned out to be a potential predictor for later complete aneurysm occlusion. Here, it might be possible to determine a threshold value that allows prediction of aneurysm occlusion with high specificity and sensitivity. As fast, applicable and easy-to-handle tool, PCC could be used for procedural monitoring and might contribute to further treatment optimization. © The Author(s) 2015.

  3. Metastatic clival chordoma: a case report of multiple extraneural metastases following resection and proton beam radiotherapy in a 5-year old boy.

    Science.gov (United States)

    Rutkowski, Martin J; Birk, Harjus S; Wood, Matthew D; Perry, Arie; Nicolaides, Theodore; Ames, Christopher P; Gupta, Nalin

    2017-05-01

    The authors report the case of a 5-year-old boy in whom extraneural metastases developed 5 years after he underwent an occipitocervical fusion and transoral approach to treat a clival chordoma without local recurrence. Following primary resection, the patient's postoperative course was complicated by recurrent meningitis secondary to CSF leak, which responded to antibiotics, and communicating hydrocephalus, for which a ventriculoperitoneal shunt was placed. The patient then underwent postoperative proton beam radiotherapy. Five years following his initial presentation, surveillance imaging revealed a new asymptomatic lung mass for which the patient underwent thoracotomy and resection of the mass. Histological examination of the lung mass revealed findings consistent with a de-differentiated chordoma, confirming extraneural metastasis from the original tumor without evidence of local recurrence. Chest wall and scalp metastases subsequently developed, and the patient was started on an adjuvant chemotherapy regimen that included imatinib and rapamycin followed by subsequent nivolumab and an EZH2 inhibitor for recurrent, disseminated disease. Despite this patient's remote and distant metastases, primary gross-total resection for chordoma remains a critical treatment objective, followed by proton beam radiotherapy. This case illustrates the importance of interval posttreatment imaging and the emerging potential to treat chordoma with molecularly targeted therapies.

  4. Clinicopathologic implications of CD8+/Foxp3+ ratio and miR-574-3p/PD-L1 axis in spinal chordoma patients.

    Science.gov (United States)

    Zou, Ming-Xiang; Guo, Ke-Miao; Lv, Guo-Hua; Huang, Wei; Li, Jing; Wang, Xiao-Bin; Jiang, Yi; She, Xiao-Ling

    2018-02-01

    Currently, little is known about the interactions between microRNAs (miRNAs) and the PD-1/PD-L1 signaling pathway in chordoma, and data discussing the role of the immune milieu in chordoma prognosis are limited. We aimed to analyze the relationship between PD-L1, miR-574-3p, microenvironmental tumor-infiltrating lymphocytes (TILs) and clinicopathological features of spinal chordoma patients. PD-L1 expression and TILs (including Foxp3 + , CD8 + , PD-1 + and PD-L1 + ) were assessed by immunohistochemistry in tumor specimens of 54 spinal chordoma patients. MiRNAs microarray and bioinformatical analysis were used to identify miRNAs potentially regulating PD-L1 expression, which were further validated by quantitative RT-PCR. miR-574-3p was identified to potentially regulate PD-L1 expression in chordoma, which inversely correlated with PD-L1. Positive PD-L1 expression on tumor cells was associated with advanced stages (P = 0.041) and TILs infiltration (P = 0.005), whereas decreased miR-574-3p level correlated with higher muscle invasion (P = 0.012), more severe tumor necrosis (P = 0.022) and poor patient survival. Importantly, a patient subgroup with PD-L1 + /miR-574-3p low chordoma phenotype was significantly associated with worse local recurrence-free survival (LRFS) (P = 0.026). PD-1 + TILs density was associated with surrounding muscle invasion (P = 0.014), and independently portended poor LRFS (P = 0.040), while PD-L1 + TILs showed tendencies of less aggressive clinical outcomes. Multivariate analysis of OS only found CD8 + /Foxp3 + ratio to be independent prognostic factor (P = 0.022). These findings may be useful to stratify patients into prognostic groups and provide a rationale for the use of checkpoint blockade therapy, possibly by administering miR-574-3p mimics, in spinal chordoma.

  5. Molecular and biochemical analyses of platelet-derived growth factor receptor (PDGFR) B, PDGFRA, and KIT receptors in chordomas.

    Science.gov (United States)

    Tamborini, Elena; Miselli, Francesca; Negri, Tiziana; Lagonigro, M Stefania; Staurengo, Samantha; Dagrada, Gian Paolo; Stacchiotti, Silvia; Pastore, Elisa; Gronchi, Alessandro; Perrone, Federica; Carbone, Antonino; Pierotti, Marco A; Casali, Paolo G; Pilotti, Silvana

    2006-12-01

    We have previously shown the presence of an activated platelet-derived growth factor (PDGF) receptor (PDGFR) B and its ligand PDGFB in a limited number of patients with clinical and radiological responses to imatinib mesylate treatment. This article describes the results of comprehensive molecular/biochemical analyses of the three receptors targeted by the drug (PDGFRB, PDGFRA, and KIT) in a series of 31 chordoma patients. The presence and activation status of PDGFRB, PDGFRA, and KIT receptors were investigated by means of immunoprecipitation and Western blot analyses complemented by immunohistochemistry, their expression level was analyzed by means of real-time PCR, and the occurrence of activating point mutations was investigated by means of cDNA sequencing. The PDGFB, PDGFA, and stem cell factor cognate ligands were investigated by reverse transcription-PCR, and gene status was assessed by fluorescence in situ hybridization. The results show that PDGFRB was highly expressed and phosphorylated, whereas PDGFRA and KIT were less expressed but phosphorylated and thus activated. These findings, together with the absence of gain-of-function mutations and the presence of the cognate ligands, strongly support the hypothesis that the activation mechanism is the autocrine/paracrine loop. No role seems to be played by gene amplification. In the light of our findings, the clinical benefit observed in chordoma patients treated with imatinib seems to be attributable to the switching off of all three receptors.

  6. Visual outcome after fronto-temporo-orbito-zygomatic approach combined with early extradural and intradural optic nerve decompression in tuberculum and diaphragma sellae meningiomas.

    Science.gov (United States)

    Mortini, Pietro; Barzaghi, Lina Raffaella; Serra, Carlo; Orlandi, Vittoria; Bianchi, Stefania; Losa, Marco

    2012-07-01

    The surgical challenge of the treatment of tuberculum (TSMs) and diaphragma sellae meningiomas (DSMs) is to preserve or improve the visual function. Extradural and intradural optic nerve decompression should reduce surgical trauma of the nerve achieving a good visual result. We reported 37 consecutive TSMs and DSMs operated through fronto-temporo-orbito-zygomatic approach with extradural unroofing of the optical canal and early intradural incision of the dural sheath. Visual data were recorded measuring the visual impairment score (VIS), the visual acuity (VA), the visual field (VF) and the postoperative improvement. A good visual outcome (VIS improved or unchanged) was obtained in 97.2% of patients (35/36). The evaluation of 72 eyes showed a good outcome (VA and VF unchanged or improved) in 98.6% (71/72 eyes). The degree of preoperative VA and VF impairment was the only factor correlating with the postoperative improvement of VA (P<.001 and P=.018) and VF defect (P<.001). Worsening of visual function occurred in 1/37 patient (2.7%). Using this surgical technique we achieved a high improvement rate of visual defects and a low frequency of worsening. Copyright © 2011 Elsevier B.V. All rights reserved.

  7. Thoracic Intradural-Extramedullary Epidermoid Tumor: The Relevance for Resection of Classic Subarachnoid Space Microsurgical Anatomy in Modern Spinal Surgery. Technical Note and Review of the Literature.

    Science.gov (United States)

    Barbagallo, Giuseppe M V; Maione, Massimiliano; Raudino, Giuseppe; Certo, Francesco

    2017-12-01

    Intradural epidermoid tumors of the spinal cord are commonly associated with spinal cord dysraphism or invasive procedures. We report the particular relationships between spinal subarachnoid compartments and thoracic intradural-extramedullary epidermoid tumor, highlighting the relevant anatomic changes that may influence microsurgery. A 40-year-old woman from compressive myelopathy owing to a thoracic epidermoid tumor extending from T3 to T4 and not associated with spina bifida, trauma, previous surgery, or lumbar spinal puncture underwent microsurgical excision. Accurate tumor membrane dissection, respecting spinal arachnoidal compartments, was performed. Reposition of a laminoplasty plateau helped in restoring thoracic spine anatomic integrity. Safe gross total tumor resection was achieved. Complete neurologic recovery as well as absence of recurrent tumor was documented at 4-year follow-up. A literature review revealed only 2 other cases of "isolated" thoracic spine epidermoid tumor. However, description of the relationship between tumor membranes and spinal subarachnoid compartments was not available in either case. A thorough knowledge of spinal subarachnoid space anatomy is helpful to distinguish between tumor membranes and arachnoidal planes and to achieve a safe and complete resection to avoid recurrences. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Analysis of the relationship between tumor dose inhomogeneity and local control in patients with skull base chordoma

    International Nuclear Information System (INIS)

    Terahara, Atsuro; Niemierko, Andrzej; Goitein, Michael; Finkelstein, Dianne; Hug, Eugen; Liebsch, Norbert; O'Farrell, Desmond; Lyons, Sue; Munzenrider, John

    1999-01-01

    Purpose: When irradiating a tumor that abuts or displaces any normal structures, the dose constraints to those structures (if lower than the prescribed dose) may cause dose inhomogeneity in the tumor volume at the tumor-critical structure interface. The low-dose region in the tumor volume may be one of the reasons for local failure. The aim of this study is to quantitate the effect of tumor dose inhomogeneity on local control and recurrence-free survival in patients with skull base chordoma. Methods and Materials: 132 patients with skull base chordoma were treated with combined photon and proton irradiation between 1978 and 1993. This study reviews 115 patients whose dose-volume data and follow-up data are available. The prescribed doses ranged from 66.6 Cobalt-Gray-Equivalent (CGE) to 79.2 CGE (median of 68.9 CGE). The dose to the optic structures (optic nerves and chiasma), the brain stem surface, and the brain stem center was limited to 60, 64, and 53 CGE, respectively. We used the dose-volume histogram data derived with the three-dimensional treatment planning system to evaluate several dose-volume parameters including the Equivalent Uniform Dose (EUD). We also analyzed several other patient and treatment factors in relation to local control and recurrence-free survival. Results: Local failure developed in 42 of 115 patients, with the actuarial local control rates at 5 and 10 years being 59% and 44%. Gender was a significant predictor for local control with the prognosis in males being significantly better than that in females (P 0.004, hazard ratio = 2.3). In a Cox univariate analysis, with stratification by gender, the significant predictors for local control (at the probability level of 0.05) were EUD, the target volume, the minimum dose, and the D 5cc dose. The prescribed dose, histology, age, the maximum dose, the mean dose, the median dose, the D 90% dose, and the overall treatment time were not significant factors. In a Cox multivariate analysis, the

  9. Updated Outcome and Analysis of Tumor Response in Mobile Spine and Sacral Chordoma Treated With Definitive High-Dose Photon/Proton Radiation Therapy

    International Nuclear Information System (INIS)

    Kabolizadeh, Peyman; Chen, Yen-Lin; Liebsch, Norbert; Hornicek, Francis J.; Schwab, Joseph H.; Choy, Edwin; Rosenthal, Daniel I.; Niemierko, Andrzej; DeLaney, Thomas F.

    2017-01-01

    Purpose: Treatment of spine and sacral chordoma generally involves surgical resection, usually in conjunction with radiation therapy. In certain circumstances where resection may result in significant neurologic or organ dysfunction, patients can be treated definitively with radiation therapy alone. Herein, we report the outcome and the assessment of tumor response to definitive radiation therapy. Methods and Materials: A retrospective analysis was performed on 40 patients with unresected chordoma treated with photon/proton radiation therapy. Nineteen patients had complete sets of imaging scans. The soft tissue and bone compartments of the tumor were defined separately. Tumor response was evaluated by the modified Response Evaluation Criteria in Solid Tumors (RECIST) and volumetric analysis. Results: With a median follow-up time of 50.3 months, the rates of 5-year local control, overall survival, disease-specific survival, and distant failure were 85.4%, 81.9%, 89.4%, and 20.2%, respectively. Eighty-four computed tomographic and magnetic resonance imaging scans were reviewed. Among the 19 patients, only 4 local failures occurred, and the median tumor dose was 77.4 GyRBE. Analysis at a median follow-up time of 18 months showed significant volumetric reduction of the total target volume (TTV) and the soft tissue target volume (STTV) within the first 24 months after treatment initiation, followed by further gradual reduction throughout the rest of the follow-up period. The median maximum percentage volumetric regressions of TTV and STTV were 43.2% and 70.4%, respectively. There was only a small reduction in bone target volume over time. In comparison with the modified RECIST, volumetric analysis was more reliable, more reproducible, and could help in measuring minimal changes in the tumor volume. Conclusion: These results continue to support the use of high-dose definitive radiation therapy for selected patients with unresected spine and sacral chordomas

  10. Benign chordoma of the sacral bone. Radiologic appearance and differential dignosis; Benignes Chordom des Os Sacrum. Radiologische Morphologie und differential-diagnostische Aspekte

    Energy Technology Data Exchange (ETDEWEB)

    Pegios, W. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Vogl, T.J. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Rausch, M. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Klein, U. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Balzer, J.O. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Hammerstingl, R. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Mack, M.G. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Felix, R. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany)

    1994-12-31

    Chordomas constitute 3-4% of all primary bony tumors [17, 20] and they arise from remnants of the notochord [4]. They can occur anywhere along the skull base and spine, where the notochord extends. 50% arise in the sacrum, 35% in the clivus and 15% in the vertebrae [17, 20]. Chordomas usually occur after the second decade with the highest incidence between the fifth and seventh decade. There is a male predominance, with roughly a 2 to 1 male-to-female ratio. Children are rarely affected [5, 25, 34]. In this article a case of a patient with a Chordoma of the sacrum is presented. After a fall on the coccyx the patient complained of recurrent and altogether increasing pain for some years. The clinical diagnosis was fracture of the coccyx with consecutive formation of callus. Finally the MRI showed a characteristically increased signal intensity in the T2-weighted spin-echo sequence (SE). With the help of MRI guided biopsy the diagnosis of a benign highly differentiated chordoma could be confirmed. (orig.) [Deutsch] Chordome stellen 3 bis 4% aller primaeren Knochentumoren dar [17, 20] und gehen von den Resten der primitiven Chorda dorsalis aus [4]. Sie koennen ueberall dort auftreten, wo embryonale Reste des Chordagewebes vorhanden sind: 50% im Sacrum, 35% im Clivus und mit 15% an den Wirbelkoepern [17, 20]. Chordome werden in der Regel nach der zweiten Lebensdekade beobachtet und erreichen ihre hoechste Inzidenz zwischen der fuenften und der siebten Lebensdekade. Sie zeigen eine Praeferenz fuer das maennliche Geschlecht mit eine Relation von ungefaehr 2:1. Kinder sind seltener betroffen [5, 25, 34]. Im folgenden soll ein Patient mit einem Chordom des Os Sacrum vorgestellt werden. Nach Sturz auf das Steissbein klagte der Patient jahrelang ueber rezidivierende und insgesamt zunehmende Schmerzsymptomatik. Die klinische Diagnose lautete Zustand nach Steissbeinfraktur mit Kallusbildung. Die schliesslich durchgefuehrte MRT zeigte eine charakteristische erhoehte

  11. Updated Outcome and Analysis of Tumor Response in Mobile Spine and Sacral Chordoma Treated With Definitive High-Dose Photon/Proton Radiation Therapy

    Energy Technology Data Exchange (ETDEWEB)

    Kabolizadeh, Peyman, E-mail: peyman.kabolizadeh@beaumont.org [Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (United States); Chen, Yen-Lin; Liebsch, Norbert [Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (United States); Hornicek, Francis J.; Schwab, Joseph H. [Department of Orthopedic Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (United States); Choy, Edwin [Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (United States); Rosenthal, Daniel I. [Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (United States); Niemierko, Andrzej; DeLaney, Thomas F. [Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (United States)

    2017-02-01

    Purpose: Treatment of spine and sacral chordoma generally involves surgical resection, usually in conjunction with radiation therapy. In certain circumstances where resection may result in significant neurologic or organ dysfunction, patients can be treated definitively with radiation therapy alone. Herein, we report the outcome and the assessment of tumor response to definitive radiation therapy. Methods and Materials: A retrospective analysis was performed on 40 patients with unresected chordoma treated with photon/proton radiation therapy. Nineteen patients had complete sets of imaging scans. The soft tissue and bone compartments of the tumor were defined separately. Tumor response was evaluated by the modified Response Evaluation Criteria in Solid Tumors (RECIST) and volumetric analysis. Results: With a median follow-up time of 50.3 months, the rates of 5-year local control, overall survival, disease-specific survival, and distant failure were 85.4%, 81.9%, 89.4%, and 20.2%, respectively. Eighty-four computed tomographic and magnetic resonance imaging scans were reviewed. Among the 19 patients, only 4 local failures occurred, and the median tumor dose was 77.4 GyRBE. Analysis at a median follow-up time of 18 months showed significant volumetric reduction of the total target volume (TTV) and the soft tissue target volume (STTV) within the first 24 months after treatment initiation, followed by further gradual reduction throughout the rest of the follow-up period. The median maximum percentage volumetric regressions of TTV and STTV were 43.2% and 70.4%, respectively. There was only a small reduction in bone target volume over time. In comparison with the modified RECIST, volumetric analysis was more reliable, more reproducible, and could help in measuring minimal changes in the tumor volume. Conclusion: These results continue to support the use of high-dose definitive radiation therapy for selected patients with unresected spine and sacral chordomas

  12. A Comparison between the Kawase and Extended Retrosigmoid Approaches (Retrosigmoid Transtentorial and Retrosigmoid Intradural Suprameatal Approaches) for Accessing the Petroclival Tumors. A Cadaveric Study.

    Science.gov (United States)

    Sharma, Mayur; Ambekar, Sudheer; Guthikonda, Bharat; Nanda, Anil

    2014-06-01

    Background The aim of our study was to compare the area of exposure at the ventral brainstem and petroclival region offered by the Kawase, retrosigmoid transtentorial (RTT), and the retrosigmoid intradural suprameatal (RISA) approaches in cadaveric models. Methods We performed 15 approaches (five each of the Kawase, RISA, and RTT approaches) on silicone-injected adult cadaver heads. Ventral brainstem and petroclival areas of exposure were measured and compared. Results The mean ventral brainstem area exposed by the Kawase approach was 55.00 ± 24.1 mm(2), significantly less than that exposed by RTT (441 ± 63.3 mm(2)) and RISA (311 ± 61 mm(2)) (p RISA (p = 0.01). The mean petroclival area of exposure through the Kawase approach was significantly smaller than that obtained through the RTT and RISA approaches (101.7 ± 545.01 mm(2), 696 ± 57.7 mm(2), and 716.7 ± 51.4 mm(2), respectively). Conclusion Retrosigmoid approaches provide a greater exposure of the brainstem and petroclival areas. The Kawase approach is ideally suited for lesions around the Meckel cave with an extension into the middle fossa. These approaches can be used in conjunction with one another to access petroclival tumors.

  13. Analysis of Patients with Myelopathy due to Benign Intradural Spinal Tumors with Concomitant Lumbar Degenerative Diseases Misdiagnosed and Erroneously Treated with Lumbar Surgery.

    Science.gov (United States)

    Lu, Kang; Wang, Hao-Kuang; Liliang, Po-Chou; Yang, Chih-Hui; Yen, Cheng-Yo; Tsai, Yu-Duan; Chen, Po-Yuan; Chye, Cien-Leong; Wang, Kuo-Wei; Liang, Cheng-Loong; Chen, Han-Jung

    2017-09-01

    When a cervical or thoracic benign intradural spinal tumor (BIST) coexists with lumbar degenerative diseases (LDD), diagnosis can be difficult. Symptoms of BIST-myelopathy can be mistaken as being related to LDD. Worse, an unnecessary lumbar surgery could be performed. This study was conducted to analyze cases in which an erroneous lumbar surgery was undertaken in the wake of failure to identify BIST-associated myelopathy. Cases were found in a hospital database. Patients who underwent surgery for LDD first and then another surgery for BIST removal within a short interval were studied. Issues investigated included why the BISTs were missed, how they were found later, and how the patients reacted to the unnecessary lumbar procedures. Over 10 years, 167 patients received both surgeries for LDD and a cervical or thoracic BIST. In 7 patients, lumbar surgery preceded tumor removal by a short interval. Mistakes shared by the physicians included failure to detect myelopathy and a BIST, and a hasty decision for lumbar surgery, which soon turned out to be futile. Although the BISTs were subsequently found and removed, 5 patients believed that the lumbar surgery was unnecessary, with 4 patients expressing regrets and 1 patient threatening to take legal action against the initial surgeon. Concomitant symptomatic LDD and BIST-associated myelopathy pose a diagnostic challenge. Spine specialists should refrain from reflexively linking leg symptoms and impaired ability to walk to LDD. Comprehensive patient evaluation is fundamental to avoid misdiagnosis and wrong lumbar surgery. Copyright © 2017 Elsevier Inc. All rights reserved.

  14. Outcomes following attempted en bloc resection of cervical chordomas in the C-1 and C-2 region versus the subaxial region: a multiinstitutional experience.

    Science.gov (United States)

    Molina, Camilo A; Ames, Christopher P; Chou, Dean; Rhines, Laurence D; Hsieh, Patrick C; Zadnik, Patricia L; Wolinsky, Jean-Paul; Gokaslan, Ziya L; Sciubba, Daniel M

    2014-09-01

    Chordomas involving the mobile spine are ideally managed via en bloc resection with reconstruction to optimize local control and possibly offer cure. In the cervical spine, local anatomy poses unique challenges, limiting the feasibility of aggressive resection. The authors present a multi-institutional series of 16 cases of cervical chordomas removed en bloc. Particular attention was paid to clinical outcome, complications, and recurrence. In addition, outcomes were assessed according to position of tumor at the C1-2 level versus the subaxial (SA) spine (C3-7). The authors reviewed cases involving patients who underwent en bloc resection of cervical chordoma at 4 large spine centers. Patients were included if the lesion epicenter involved the C-1 to C-7 vertebral bodies. Demographic data and details of surgery, follow-up course, exposure to adjuvant therapy, and complications were obtained. Outcome was correlated with presence of tumor in C1-2 versus subaxial spine via a Student t-test. Sixteen patients were identified (mean age at presentation 55 ± 14 years). Seven cases (44%) cases involved C1-2, and 16 involved the subaxial spine. Median survival did not differ significantly different between the C1-2 (72 months) and SA (60 months) groups (p = 0.65). A combined (staged anteroposterior) approach was used in 81% of the cases. Use of the combined approach was significantly more common in treatment of subaxial than C1-2 tumors (100% vs 57%, p = 0.04). En bloc resection was attempted via an anterior approach in 6% of cases (C1-2: 14.3%; SA: 0%; p = 0.17) and a posterior approach in 13% of cases (C1-2: 29%; SA: 0%; p = 0.09). The most commonly reported margin classification was marginal (56% of cases), followed by violated (25%) and wide (19%). En bloc excision of subaxial tumors was significantly more likely to result in marginal margins than excision of C1-2 tumors (C1-2: 29%; SA: 78%; p = 0.03). C1-2 tumors were associated with significantly higher rates of

  15. SU-F-T-221: An Assessment of the Potential for Improved Local Control of Skull- Base Chordomas Via Reduction of the Proton Beam Range Uncertainty

    Energy Technology Data Exchange (ETDEWEB)

    Muller, L; Soldner, A; Kirk, M; Fager, M; Solberg, T; Robert, L; Dolney, D [University of Pennsylvania, Philadelphia, PA (United States)

    2016-06-15

    Purpose: The beam range uncertainty presents a special challenge for proton therapy. Novel technologies currently under development offer strategies to reduce the range uncertainty [1,2]. This work quantifies the potential advantages that could be realized by such a reduction for dosimetrically challenging chordomas at the base of skull. Therapeutic improvement was assessed by evaluating tumor control probabilities (TCP) and normal tissue complication probabilities (NTCP). Methods: Treatment plans were made for a modulated-scanned proton delivery technique using the Eclipse treatment planning system. The prescription dose was 7920 cGy to the CTV. Three different range uncertainty scenarios were considered: 5 mm (3.5% of the beam range + 1 mm, representing current clinical practice, “Curr”), 2 mm (1.3%), and 1 mm (0.7%). For each of 4 patients, 3 different PTVs were defined via uniform expansion of the CTV by the value of the range uncertainty. Tumor control probability (TCP) and normal tissue complication probabilities (NTCPs) for organs-at-risk (OARs) were calculated using the Lyman-Kutcher-Burman[3] formalism and published model parameters [ref Terahara[4], quantec S10, Burman Red Journal v21 pp 123]. Our plan optimization strategy was to achieve PTV close to prescription while maintaining OAR NTCP values at or better than the Curr plan. Results: The average TCP values for the 5, 2, and 1 mm range uncertainty scenarios are 51%, 55% and 65%. The improvement in TCP for patients was between 4 and 30%, depending primarily on the proximity of the GTV to OAR. The average NTCPs for the brainstem and cord were about 4% and 1%, respectively, for all target margins. Conclusion: For base of skull chordomas, reduced target margins can substantially increase the TCP without increasing the NTCP. This work demonstrates the potential significance of a reduction in the range uncertainty for proton beams.

  16. Intradural spinal neurocysticercosis: case illustration

    Directory of Open Access Journals (Sweden)

    Balderrama Jorge

    2017-12-01

    Full Text Available Introduction: Neurocysticercosis (NCC is a common parasitic infection of the central nervous system caused by the larvae of the Taenia solium. Spinal cord involvement is very uncommon. Clinical case: A female patient with a history of NCC presented with chronic and recurrent headache associated with motor and sensory deficit, which develops tonic-clonic convulsion, with spatial disorientation. She also had intracranial hypertension syndrome, meningitis syndrome, and pyramidal sygns suggestive of spinal NCC. Conclusions: Neurocysticercosis usually occurs in developing countries and should be considered as a differential diagnosis of neurological diseases. Early diagnosis and treatment are mandatory, as well as education to the community to primary prevention.

  17. Sacrococcygeal chordoma in a 9-year-old boy Cordoma sacrococígeo em um menino de 9 anos de idade

    Directory of Open Access Journals (Sweden)

    Lúcia de Noronha

    1995-09-01

    Full Text Available A case of sacrococcygeal chordoma in a 9-year-old boy is presented. The symptoms at presentation were pain in both legs and sacrococcygeal region for the last two years that increased in the last four weeks irradiating mainly to the left leg. X-ray and CT scan examinations of the lumbar region revealed an expansive process in the coccygeal region with multiple calcifications and a partially eroded coccyx. There was no invasion of the retroperitoneum and regional lymph nodes. A biopsy was performed and showed cords and nests of cells with large cytoplasm, sometimes vacuolated, nuclei with moderate pleomorphism and clumped chromatin. Immunohistochemistry with avidin-biotin peroxidase technique showed positivity for CK, S-100 protein, CEA, vimentin and to EMA. Chordomas are a distinctly uncommon neoplasm in the first two decades of life, specially in the sacrococcygeal region. They have an aggressive behavior. Treatment of choice is complete resection.Os autores apresentam um caso de cordoma sacroccígeo em um menino de 9 anos de idade. O paciente foi admitido no hospital com história de dor na região sacral e nos membros inferiores com dois anos de evolução, piorando nas últimas quatro semanas. O exame físico revelou atrofia muscular moderada em ambos os membros inferiores, diminuição do reflexo patelar e presença do sinal de Lasègue à esquerda. Os exames de imagem da região lombar mostraram um processo expansivo na região sacrococcígea com erosão parcial do coccix e focos de calcificação, sem evidência de metástases para linfonodos regionais. Foi realizada biópsia diagnóstica que mostrou neoplasia formada por cordões e ninhos de células de citoplasma amplo, por vezes vacuolado, com núcleos moderadamente pleomórficos com cromatina grumosa. O estudo imuno-histoquímico revelou positividade para CK, proteína S-100, CEA, vimentina e EMA. Cordomas são tumores raros que representam em torno de 2% de todas as neoplasias

  18. Improvement of the local control of spinal chordomas treated by surgery and targeted irradiation (CyberKnife{sup R}) on hypoxic cells marked with {sup 18}F-FMiso; Amelioration du controle local des chordomes du rachis traites par chirurgie et une irradiation (CyberKnife{sup R}) ciblee sur les cellules hypoxiques marquees au {sup 18}F-FMiso

    Energy Technology Data Exchange (ETDEWEB)

    Mammar, H.; Kerrou, K.; Bondiau, P.Y.; Angellier, G.; Thariat, J.; Benezery, K.; Heroult, J.; Leysalle, A.; Gerard, J.P. [Centre Antoine-Lacassagne, Nice (France); Talbot, J.N. [Service de medecine nucleaire, Hopital Tenon, AP-HP, Paris (France)

    2011-10-15

    The authors report and comment the treatment of two women suffering from a recurring cervical spine chordoma. Each patient had a first PET (positron emission tomography) with {sup 18}F-fluorodeoxyglucose to assess the hyper-metabolic component and PET with {sup 18}F-FMiso to quantify the hypoxic component within the lesion. This last PET allows a non-invasive quantification of the hypoxic component which is potentially radio-resistant in cervical spine chordomas. It also allows an intelligent dose increase to improve the local control rate. Short communication

  19. Circulating vascular endothelial growth factor (VEGF) as predictive factor of progression-free survival in patients with advanced chordoma receiving sorafenib: an analysis from a phase II trial of the french sarcoma group (GSF/GETO)

    Science.gov (United States)

    Lebellec, Loic; Bertucci, François; Tresch-Bruneel, Emmanuelle; Bompas, Emmanuelle; Toiron, Yves; Camoin, Luc; Mir, Olivier; Laurence, Valerie; Clisant, Stephanie; Decoupigny, Emilie; Blay, Jean-Yves; Goncalves, Anthony; Penel, Nicolas

    2016-01-01

    Background Patients with advanced chordoma are often treated with tyrosine kinase inhibitors without any predictive factor to guide decision. We report herein an ancillary analysis of the the Angionext phase II trial (NCT 00874874). Results From May 2011 to January 2014, 26 were sampled. The 9-month PFS rate was 72.9% (95%-CI: 45.9-87.9). During sorafenib treatment, a significant increase in PlGF (18.4 vs 43.8 pg/mL, p1.04 ng/mL (HR=12.5, 95%-CI: 1.37-114, p=0.025) and VEGF at D7 >1.36 ng/mL (HR=10.7, 95%-CI: 1.16-98, p=0.037) were associated with shorter PFS. The 9-month PFS rate was 92.3% (95%-CI: 56.6-98.9) when VEGF at D1 was ≤1.04 ng/mL versus 23.3% (95%-CI: 1.0-63.2) when >1.04 ng/mL. Patients and Methods Chordoma patients were treated with sorafenib 800 mg/day for 9 months, unless earlier occurrence of progression or toxicities. Six biomarkers (sE-Selectin, VEGF, VEGF-C, placental growth factor (PlGF), Thrombospondin, Stem Cell Factor (SCF)) were measured at baseline (day 1: D1) and day 7 (D7). Conclusion High levels of VEGF was associated with poor outcome. PMID:27659533

  20. Fatores prognósticos no tratamento dos tumores intradurais extramedulares: estudo de 44 casos Prognostic factors in the treatment of the intradural extramedullary tumors: a study of 44 cases

    Directory of Open Access Journals (Sweden)

    Daniel Monte-Serrat Prevedello

    2003-06-01

    Full Text Available Entre 1993 e 1999 foram estudados 44 pacientes que preencheram requisitos protocolares de seguimento adequado e que foram submetidos à ressecção de processo expansivo extramedular intradural. Os pacientes eram constituídos por 43,2% de mulheres e por 56,8% de homens. A média da idade foi 32,9 anos. A localização da lesão foi mais comum na região torácica, com 45,5% dos casos, seguida da lombar com 18,2%. A extensão tumoral variou de 1 a 7 segmentos vertebrais, com uma média de 2,5 níveis. O schwanoma foi o tipo histológico mais comum com 65,9%, seguido dos meningiomas com 20,5%. Houve 2 casos de neurofibroma e 1 caso de paraganglioma, cisto neuroentérico, metástase e schwanoma maligno. A evolução foi de melhora em 56,8%, estabilidade em 31,8% e de piora em 11,4%. Não houve mortalidade relacionada ao procedimento cirúrgico. Todos os casos de piora tiveram ressecção total e eram localizados no segmento torácico. A ressecção total é a modalidade ideal de tratamento cirúrgico. No entanto, no nível torácico, onde predominam as peculiaridades de irrigação da medula, a morbidade cirúrgica pode ser mais elevada (p=0,014.Between 1993 and 1999, 44 patients submitted to resection of an expansible intradural extramedullary lesion who filled protocol requirements of appropriate follow up were studied. Patients were constituted by 43.2% female and 56.8% male. The mean age was 32.9 years old. Lesion most common location was at the thoracic spine, with 45.5% of the cases, followed by the lumbar level with 18.2%. Tumor extension varied from 1 to 7 vertebral segments, with an average of 2.5 levels. Schwannoma, with 65,9% of the cases, was the most frequent lesion, followed by meningioma with 20.5%. There were 2 cases of neurofibroma and 1 case of paraganglioma, neuroenteric cyst, metastasis and malignant schwannoma. The evolution was of improvement in 56.8%, stability in 31.8% and of worsening in 11.4%. There was no mortality

  1. A diagnostic trap potentially lethal: chordomas uncovered by C3 large osseous destruction inconspicuous on bis-phosphonates-({sup 99}Tc) bone scintigraphy; Un piege diagnostique potentiellement letal: chordome revele par une vaste lesion lytique de C3 normofixante a la scintigraphie osseuse aux bisphosphonates-({sup 99}Tc)

    Energy Technology Data Exchange (ETDEWEB)

    Paycha, F.; Ramadan, A.; Akrout, L. [Hopital Louis Mourier, Unite de Medecine Nucleaire, 92 - Colombes (France); Dion, E. [Hopital Louis Mourier, Service de Radiologie, 92 - Colombes (France); Grossin, M. [Hopital Louis Mourier, Service d' Anatomie Pathologique, 92 - Colombes (France); Gazals-Hatem, D. [Hopital Beaujon, Service d' Anatomie Pathologique, 92 - Clichy (France)

    2006-07-15

    The authors describe a case report of a brachialgy uncovering a solitary bone destruction of C3 associated with epidural involvement. CT and MRI properly depicted the lesion and the loco-regional extension but failed to entertain the diagnosis of the causative tumor, a chordoma. Bis-phosphonates-({sup 99m}Tc) bone scintigraphy proved unconspicuous. Diagnosis was eventually established from the resection specimen, after decision of C3 spondyl-ectomy, C4 hemi-superior spondyl-ectomy and epidural extension excision. Immunohistochemistry study of the material was decisive in identifying the tumor. Cervical chordoma is a tricky diagnosis. Discussion underscores that the crux of diagnostic process is the double discrepancy firstly between extra-osseous and intra-osseous tumoral expansion, secondly between large tumoral mass obvious on multi-slice morphological imaging and negativity of bone scintigraphy, SPECT study included. Among the armamentarium of conventional scintigraphies and PET studies, methionine-({sup 11}C) PET emerges as the most promising anatomo-metabolic procedure to assess loco-regional tumoral expansion, tumoral viability, therapy efficiency, and, potentially, to search for metastases in chordoma. (author)

  2. SU-F-T-203: High Dose Definitive Proton Therapy with Integrated Boost to Hypoxic Subvolumes Determined by [18F]-Fluoromisonidazole (FMISO) PET/CT for Patients with Recurrent Chordomas Using Intensity Modulated Proton Therapy (IMPT)

    Energy Technology Data Exchange (ETDEWEB)

    Yan, S; Broussard, G; De, K; Bernstein, Amorim; Wang, Y [Massachusetts General Hospital and Harvard Medical School, Department of Radiation Oncology, Boston, MA (United States)

    2016-06-15

    Purpose: Recurrent chordomas are difficult to control locally. This dosimetric study investigates the feasibility of dose escalation to hypoxic regions, visualized on FMISO-PET, while respecting the dose constraints to the neighboring normal tissues/organs. We propose to deliver a higher dose to the areas of hypoxia (84.5Gy) using IMPT with the goal of improving local control. Methods: We currently have four patients with hypoxic subvolumes (HSV) greater than 10cc from the FMISO-PET image. The HSV was delineated based on the standardized uptake values of greater than 1.4 times of the muscle mean. Gross tumor volume (GTV) was delineated using planning CT with the assistance of MRI fusion. The dose scheme is 50.4Gy RBE to CTV in 1.8Gy fractions, followed by an integrated boost of 27.0Gy RBE to GTV in 1.8Gy fractions and 34.5Gy RBE to HSV in 2.3Gy fractions. IMPT integrated boost plans were optimized with multi-criteria optimization (MCO). Posterior-anterior beam angles were used for these plans. We also propose using two posterior oblique fields to boost HSV to spare the skin folding. A medium spot size with 8mm to 15 mm (σ) in air at isocenter with energies from 220 MeV down to 90 MeV was used. Aperture was used for the medium spot size. A small spot size of 2.5 mm to 4.5 mm (σ) in air at isocenter with energies from 240 MeV down to 70 MeV was also proposed. Target coverage and dose to OARs were evaluated. Results: For the sacral chordoma patient that has been planned, the target homogeneity index is 3.2% for HSV, 55.9% for CTV and 11.9% for GTV. The max dose is 77GyRBE to rectum, 86.2GyRBE to sacral nerves and 73.9GyRBE to cauda equina. Conclusion: IMPT with integrated high dose boost to HSV determined from FMISO PET image is feasible. OAR dose constraints were met.

  3. Lack of efficacy of an intradural somatic-to-autonomic nerve anastomosis (Xiao procedure) for bladder control in children with myelomeningocele and lipomyelomeningocele: results of a prospective, randomized, double-blind study.

    Science.gov (United States)

    Tuite, Gerald F; Polsky, Ethan G; Homsy, Yves; Reilly, Margaret A; Carey, Carolyn M; Parrish Winesett, S; Rodriguez, Luis F; Storrs, Bruce B; Gaskill, Sarah J; Tetreault, Lisa L; Martinez, Denise G; Amankwah, Ernest K

    2016-08-01

    OBJECTIVE Xiao et al. and other investigators have studied an intradural somatic-to-autonomic (e.g., L-5 to S3-4) nerve transfer as a method to create a reflex arc to allow bladder emptying in response to cutaneous stimulation (the Xiao procedure). In previous clinical studies of patients with spinal dysraphism who underwent the Xiao procedure, high success rates (70%-85%) were reported for the establishment of a "skin-CNS-bladder" reflex arc that allows spontaneous, controlled voiding in children with neurogenic bladder dysfunction. However, many of these studies did not use blinded observers, did not have control groups, and/or featured only limited follow-up durations. METHODS A randomized, prospective, double-blind trial was initiated in March 2009, enrolling children with myelomeningocele (MM), lipomyelomeningocele (LMM), and neurogenic bladder dysfunction who were scheduled for spinal cord detethering (DT) for the usual indications. At the time of DT, patients were randomized between 2 arms of the study: half of the patients underwent a standard spinal cord DT procedure alone (DT group) and half underwent DT as well as the Xiao procedure (DT+X group). Patients, families, and study investigators, all of whom were blinded to the surgical details, analyzed the patients' strength, sensory function, mobility, voiding, and urodynamic bladder function before surgery and at regular intervals during the 3-year follow-up. RESULTS Twenty patients were enrolled in the study: 10 underwent only DT and the other 10 underwent DT+X. The addition of the Xiao procedure to spinal cord DT resulted in longer operative times (p = 0.024) and a greater chance of wound infection (p = 0.03). Patients in both treatment arms could intermittently void or dribble small amounts of urine (bladder capacity) in response to scratching in dermatomes T-9 through S-2 using a standardized protocol, but the voiding was not reproducible and the volume voided was not clinically useful in any patient

  4. Adaptation of proton total dose with respect to dosimetric parameters within the frame of treatment of skull base or upper cervical spine chordomas; Adaptation de la dose totale de protons en fonction des parametres dosimetriques dans le cadre du traitement des chordomes de la base du crane et du rachis cervical haut

    Energy Technology Data Exchange (ETDEWEB)

    Hemery, C.G.; Mazeron, J.J.; Feuvret, L. [Groupe hospitalier Pitie-Salpetriere (AP-HP), 75 - Paris (France); Calugaru, V.; Bolle, S.; Habrand, J.L.; Datcharty, J.; Alapetite, C.; Dendale, R.; Feuvret, L. [Institut Curie-Centre de protontherapie d' Orsay, 91 (France); Habrand, J.L.; Datcharty, J. [Institut Gustave-Roussy, 94 - Villejuif (France); Noel, G. [Centre Paul-Strauss, 67 - Strasbourg (France)

    2010-10-15

    The authors report the study of the feasibility of a photon-proton irradiation protocol with a dose adaptation with respect to dosimetric factors for patients suffering form a skull base and upper cervical spine chordoma. Sixty patients have been treated between May 2006 and June 2008 with a combination of high energy photons and protons. As five tumours have locally relapsed and one at distance, the authors comment the local control rates, the number of attained cranial nerves, the value of the macroscopic tumour volume, the survival rate without relapse in terms of multifactorial of uni-factorial analysis. Short communication

  5. Novel targeted therapies in chordoma: an update

    OpenAIRE

    Di Maio, Salvatore; Yip, Stephen; Al Zhrani, Gmaan A; Alotaibi, Fahad E; Al Turki, Abdulrahman; Kong, Esther; Rostomily, Robert C

    2015-01-01

    Salvatore Di Maio,1 Stephen Yip,2 Gmaan A Al Zhrani,3,4 Fahad E Alotaibi,3,4 Abdulrahman Al Turki,3,4 Esther Kong,2 Robert C Rostomily5 1Division of Neurosurgery, Jewish General Hospital, McGill University, Montreal, QC, 2Department of Pathology and Laboratory Medicine, Vancouver General Hospital, University of British Columbia, Vancouver, BC, Canada; 3National Neuroscience Institute, Department of Neurosurgery, King Fahad Medical City, Riyadh, Saudi Arabia; 4Department of Neurology...

  6. Advanced chordoma treated by first-line molecular targeted therapies: Outcomes and prognostic factors. A retrospective study of the French Sarcoma Group (GSF/GETO) and the Association des Neuro-Oncologues d'Expression Française (ANOCEF).

    Science.gov (United States)

    Lebellec, Loïc; Chauffert, Bruno; Blay, Jean-Yves; Le Cesne, Axel; Chevreau, Christine; Bompas, Emmanuelle; Bertucci, François; Cupissol, Didier; Fabbro, Michel; Saada-Bouzid, Esma; Duffaud, Florence; Feuvret, Loïc; Bonneville-Levard, Alice; Bay, Jacques-Olivier; Vauleon, Elodie; Vinceneux, Armelle; Noel, Georges; Penel, Nicolas; Mir, Olivier

    2017-07-01

    To assess the role of first-line Molecular Targeted Therapies (MTTs) in Advanced chordoma (AC) patients. Retrospective study of 80 patients treated between January 2004 and December 2015 at 15 major French Sarcoma or Neurooncology Centres. The sex ratio M/F was 46/34. The median age was 59 (6-86) years. The primary sites were the sacrum (50, 62.5%), mobile spine (12, 15.0%), and skull base (18, 22.5%). Metastases were present in 28 patients (36.0%). The first line of MTTs consisted of imatinib (62, 77.5%), sorafenib (11, 13.7%), erlotinib (5, 6.3%), sunitinib (1, 1.2%) and temsirolimus (1, 1.2%). The reported responses were: partial response (5, 6.3%), stable disease (58, 72.5%), or progressive disease (10, 12.5%). Symptomatic improvement was seen in 28/66 assessable patients (42.4%) and was associated with an objective response occurrence (p = 0.005), imatinib (p = 0.020) or erlotinib use (p = 0.028). The median progression-free survival (PFS) was 9.4°months (95% CI, [6.8-16.1]). Two independent factors of poor prognosis for PFS were identified: a skull-based primary location (HR = 2.5, p = 0.019), and the interval between diagnosis and MTT of <52months (HR = 2.8, p < 0.001). The median overall survival (OS) was 4.4°years (95% CI, [3.8-5.6]). Four independent factors of poor prognosis for OS were identified: the presence of liver metastases (HR = 13.2, p < 0.001), pain requiring opioids (HR = 2.9, p = 0.012), skull-based primary location (HR = 19.7, p < 0.001), and prior radiotherapy (photon alone) (HR = 2.5, p = 0.024). The PFS and OS did not significantly differ between the MTT. The prognostic factors identified require validation in an independent database but are potently useful to guide treatment decisions and design further clinical trials. Copyright © 2017 Elsevier Ltd. All rights reserved.

  7. Distinguishing benign notochordal cell tumors from vertebral chordoma

    Energy Technology Data Exchange (ETDEWEB)

    Yamaguchi, Takehiko [Sapporo Medical University School of Medicine, Department of Surgical Pathology, Sapporo, Hokkaido (Japan); Iwata, Jun [Kochi Health Science Center, Department of Laboratory Medicine, Kochi, Kochi (Japan); Sugihara, Shinsuke [Kochi Health Science Center, Department of Orthopaedic Surgery, Kochi, Kochi (Japan); McCarthy, Edward F. [The Johns Hopkins Hospital, Department of Pathology, Baltimore, MD (United States); Karita, Michiaki; Murakami, Hideki; Kawahara, Norio; Tsuchiya, Hiroyuki; Tomita, Katsuro [Kanazawa University, Department of Orthopaedic Surgery, Kanazawa, Ishikawa (Japan)

    2008-04-15

    The objective was to characterize imaging findings of benign notochordal cell tumors (BNCTs). Clinical and imaging data for 9 benign notochordal cell tumors in 7 patients were reviewed retrospectively. Conventional radiographs (n = 9), bone scintigrams (n = 2), computed tomographic images (n = 7), and magnetic resonance images (n = 8) were reviewed. Eight of the 9 lesions were stained with hematoxylin-eosin and microscopically examined. There were 3 male and 4 female patients with an age range of 22 to 55 years (average age, 44 years). Two patients had two lesions at different sites. The lesions involved the cervical spine in 4 patients, the lumbar spine in 2, the sacrum in 2, and the coccyx in 1. The most common symptom was mild pain. The lesions of 2 patients were found incidentally during imaging studies for unrelated conditions. Five patients underwent surgical procedures. One patient died of surgical complications. All other patients have been well without recurrent or progressive disease for 13 to 84 months. Radiographs usually did not reveal significant abnormality. Five lesions exhibited subtle sclerosis and 1 showed intense sclerosis. Technetium bone scan did not reveal any abnormal uptake. Computed tomography images had increased density within the vertebral bodies. The lesions had a homogeneous low signal intensity on T1-weighted magnetic resonance images and a high intensity on T2-weighted images without soft-tissue mass. Microscopically, lesions contained sheets of adipocyte-like vacuolated chordoid cells without a myxoid matrix. Benign notochordal cell tumors may be found during routine clinical examinations and do not require surgical management unless they show extraosseous disease. These tumors should be recognized by radiologists, pathologists, and orthopedic surgeons to prevent operations, which usually are extensive. (orig.)

  8. Severe Intradural Lumbar Disc Herniation with Cranially Oriented Free Fragment Migration.

    Science.gov (United States)

    Tempel, Zachary; Zhu, Xiao; McDowell, Michael M; Agarwal, Nitin; Monaco, Edward A

    2016-08-01

    Intrathecal disc herniation is a rare but serious condition that has the potential to generate devastating neurologic deficits. We present a case of a 56-year-old man who developed cauda equina syndrome after several episodes of severe Valsalva maneuver. The patient was found to have developed subacute urinary retention and leg weakness. Magnetic resonance imaging findings were concerning for an unusual-appearing lesion extending cranially at L2-3. Urgent decompression via an L2 laminectomy, exploration, and subsequent discectomy was performed. The patient recovered exceptionally well, regaining bladder function and ultimately being able to ambulate without assistance. Cranially extending intrathecal disc herniations are a rare phenomenon and exceptionally uncommon above L3. The clinician should have a high level of suspicion for herniation when looking at the clinical and historical information consistent with such a diagnosis even in the presence of ambiguous imaging findings. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Gunshot Wound in Lumbar Spine with Intradural Location of a Bullet

    Directory of Open Access Journals (Sweden)

    G. Bordon

    2014-01-01

    Full Text Available The presence of a migratory bullet in the spinal canal after a gunshot injury is a rare finding, specially without causing permanent neurologic damage. We present the case of a patient who suffered a gunshot wound with an entry point in the posterior arc of L2-L3 and a migratory bullet detected at the level of L5-S1 in the CT scan. The patient complained about intense headache, dizziness, and variable sensitive impairment in lower legs apparently depending on the patient’s position in bed. We decided to remove the bullet in order to prevent the delayed neurological damage and lead toxicity. We discuss technical details of this surgery.

  10. Bilateral retro-auricular dermal sinus tracts with intradural extension. Case report.

    Science.gov (United States)

    Nejat, Farideh; Dias, Mark S; Eftekhar, Behzad; Roodsari, Nahid Nasri; Hamidi, Saiid

    2003-07-01

    The authors describe a previously unreported malformation involving paired, bilaterally symmetrical dermal sinus tracts in the retro-auricular area, both of which passed through the asterion and posterior fossa dura mater to end intracranially. Cranial dermal sinus tracts are congenital lesions that virtually always originate from the midline scalp posteriorly at the external occipital protuberance, anteriorly at the nasion or along the nasal dorsum, or in the posterior parietal midline. A lateral origin is extremely rare, and intracranial extension of a lateral dermal sinus tract has not been reported previously. The authors propose an embryological mechanism to explain the origin of this rare malformation and discuss its management.

  11. Uterine carcinosarcoma associated with pelvic radiotherapy for sacral chordoma: A case report

    Directory of Open Access Journals (Sweden)

    Korhan Kahraman

    2012-03-01

    Conclusion: In uterine masses seen in patients with history of irradiation to the pelvic field, the probability of uterine sarcomas should always be kept in mind. These tumors may occur simultaneously with recurrence of primary tumor previously treated by adjuvant radiation therapy.

  12. High expression of Sam68 in sacral chordomas is associated with worse clinical outcomes

    OpenAIRE

    Wen, Hai; Li, Pengzhi; Ma, Hong; Zheng, Jiaoyun; Yu, Yipin; Lv, Guohua

    2017-01-01

    Hai Wen,1,* Pengzhi Li,1,* Hong Ma,1 Jiaoyun Zheng,2 Yipin Yu,1 Guohua Lv1 1Department of Spine Surgery, The Second Xiangya Hospital of Central South University, Changsha, Hunan, China; 2Department of Pathology, The Second Xiangya Hospital of Central South University, Changsha, Hunan, China *These authors contributed equally to this work Abstract: Src-associated in mitosis of 68 kDa (Sam68), also known as KHDRBS1 (KH domain-containing, RNA-binding, signal transduction-associated 1), is a m...

  13. Combat-related intradural gunshot wound to the thoracic spine: significant improvement and neurologic recovery following bullet removal.

    Science.gov (United States)

    Louwes, Thijs M; Ward, William H; Lee, Kendall H; Freedman, Brett A

    2015-02-01

    The vast majority of combat-related penetrating spinal injuries from gunshot wounds result in severe or complete neurological deficit. Treatment is based on neurological status, the presence of cerebrospinal fluid (CSF) fistulas, and local effects of any retained fragment(s). We present a case of a 46-year-old male who sustained a spinal gunshot injury from a 7.62-mm AK-47 round that became lodged within the subarachnoid space at T9-T10. He immediately suffered complete motor and sensory loss. By 24-48 hours post-injury, he had recovered lower extremity motor function fully but continued to have severe sensory loss (posterior cord syndrome). On post-injury day 2, he was evacuated from the combat theater and underwent a T9 laminectomy, extraction of the bullet, and dural laceration repair. At surgery, the traumatic durotomy was widened and the bullet, which was laying on the dorsal surface of the spinal cord, was removed. The dura was closed in a water-tight fashion and fibrin glue was applied. Postoperatively, the patient made a significant but incomplete neurological recovery. His stocking-pattern numbness and sub-umbilical searing dysthesia improved. The spinal canal was clear of the foreign body and he had no persistent CSF leak. Postoperative magnetic resonance imaging of the spine revealed contusion of the spinal cord at the T9 level. Early removal of an intra-canicular bullet in the setting of an incomplete spinal cord injury can lead to significant neurological recovery following even high-velocity and/or high-caliber gunshot wounds. However, this case does not speak to, and prior experience does not demonstrate, significant neurological benefit in the setting of a complete injury.

  14. General aspects of sacrococcygeal tumors

    International Nuclear Information System (INIS)

    Mosquera Betancourt, Gretel; Hernández González, Erick Héctor

    2015-01-01

    Background: tumors arising from sacrococcygeal region are weird and represent from one to seven percent of primary spinal tumors. However diagnosis and treatment are a challenge for the surgical staff because of the anatomic relations complexity and the more frequent lesions biological features. Objective: a review of the literature was done to update and systematize the knowledge about general aspects of sacrococcygeal tumors. Development: they are more frequent in male young adults, unspecific low back pain is the onset symptom and muscle weakness, sense deficit and sphincter disturbances join up later progressively. They can be epidural or intradural, primary or secondary, neoplastics or not and they could arise from soft tissues or bone structure. Age and location most be considered to evaluate malignant potential. CT scan and magnetic resonance imaging are the confirmatory tests and allow surgical approach selection. The most frequent affections are chordoma, aneurismal bone cyst and giant cell tumor. Radiotherapy and chemotherapy can support surgery according to histology. Conclusions: precocious diagnosis needs to be suspected before unspecific symptoms. Sacrococcygeal tumors treatment most be multidisciplinary and multimodal to assure better prognosis and patient´s life expectancy increasing. (author)

  15. Spinal Rosai–Dorfman disease: Case report of a rare disorde

    Directory of Open Access Journals (Sweden)

    A. Elsotouhy

    2015-12-01

    Conclusion: This is a rare case of spinal Rosai–Dorfman Disease with epidural and intradural components causing cord compression. To our knowledge, this represents the first case of combined epidural and intradural extramedullary lesions in the literature.

  16. Prevention: Exercise

    Medline Plus

    Full Text Available ... Osteoporosis Spondylolysis and Spondylolysthesis Injuries Spinal Cord Injuries Sports Injuries Whiplash and Whiplash Associated Disorder Infections & Tumors Spinal Infections Spinal Tumors Extradural and Intradural ...

  17. Prevention: Exercise

    Medline Plus

    Full Text Available ... Injuries Sports Injuries Whiplash and Whiplash Associated Disorder Infections & Tumors Spinal Infections Spinal Tumors Extradural and Intradural Tumors TREATMENTS Assessment ...

  18. Prevention: Exercise

    Medline Plus

    Full Text Available ... Sports Injuries Whiplash and Whiplash Associated Disorder Infections & Tumors Spinal Infections Spinal Tumors Extradural and Intradural Tumors TREATMENTS Assessment Tools Lumbar ...

  19. Conus Medullaris Arachnoid Cyst Presenting as Cauda Equina Syndrome.

    Science.gov (United States)

    Sharif, Salman; Afsar, Afifa; Qadeer, Mohsin

    2017-01-01

    Intradural arachnoid cysts are a rare cause of spinal cord and nerve root compression. Primarily, they are present in the thoracic region posteriorly. We report a 25-year-old man who had an intradural arachnoid cyst at the level of conus medullaris presenting with cauda equina syndrome, which is very rare.

  20. Bladder compliance after posterior sacral root rhizotomies and anterior sacral root stimulation

    NARCIS (Netherlands)

    Koldewijn, E. L.; van Kerrebroeck, P. E.; Rosier, P. F.; Wijkstra, H.; Debruyne, F. M.

    1994-01-01

    To evaluate the effects of central detrusor denervation on bladder compliance, we studied 27 patients with complete suprasacral spinal cord injury in whom intradural posterior sacral root rhizotomies from S2 to S5 in combination with implantation of an intradural Finetech-Brindley bladder stimulator

  1. Cervical arachnoid cyst mimicking the cause of cervical radiculopathy: A case report

    Directory of Open Access Journals (Sweden)

    Takashi Kobayashi, MD, PhD

    2017-12-01

    Full Text Available We herein present a case involving an asymptomatic cervical intradural arachnoid cyst that was suspected to be the cause of the patient's radicular pain. Magnetic resonance imaging showed a small, round intradural mass beside the cervical nerve root. An arachnoid cyst of this shape has not been previously reported. Intraoperative examination revealed that the cyst was not compressing the nerve root and was asymptomatic. Intradural spinal surgery is sometimes associated with serious complications. The knowledge that arachnoid cysts of this shape are asymptomatic can help to avoid unnecessary surgery.

  2. PATTERNS OF SEVEN AND COMPLICATED MALARIA IN CHILDREN

    African Journals Online (AJOL)

    GB

    neurological tuberculosis and includes cases of arachnoiditis, intradural spinal tuberculoma or granuloma, and spinal cord complications of ... Spinal Injury Association (ASIA). Cerebrospinal fluid analysis showed xanthochromic fluid that ... countries, it affects elderly people due to reactivated disease. However patients from.

  3. SURGICAL OUTCOME OF CERVICAL AND FORAMEN MAGNUM INTRA DURAL AND INTRA MEDULLARY TUMOURS

    OpenAIRE

    Kumar Babu; Deekshanti Narayan; Biju; Manas; Vijaya Saradhi; Pathapati Rama; Madhavalu

    2014-01-01

    : INTRODUCTION: Spinal tumors account for only approximately 5-15% of the nervous system neoplasms. Technical advances in imaging and surgical procedures have brought about significant better clinical results in the last 2 decades. We also evaluated surgical and functional outcomes in patients having cervical intradural tumors including tumors at foramen magnum. METHODS: All patients who underwent surgical treatment for cervical intradural tumors from foramen magnum to C7,...

  4. Giant Vertebral Notochordal Rest: Magnetic Resonance and Diffusion Weighted Imaging Findings

    International Nuclear Information System (INIS)

    Oner, Ali Yusuf; Akpek, Sergin; Tali, Turgut; Ucar, Murat

    2009-01-01

    A giant vertebral notochordal rest is a newly described, benign entity that is easily confused with a vertebral chordoma. As microscopic notochordal rests are rarely found in adult autopsies, the finding of a macroscopic vertebral lesion is a new entity with only seven previously presented cases. We report here radiological findings, including diffusion weighted images, of a patient with a giant notochordal remnant confined to the L5 vertebra, with an emphasis on its distinction from a chordoma

  5. Transdural herniated lumbar disc disease with muscle patch for closure of durotomy - A Brief review of literature.

    Science.gov (United States)

    Huliyappa, Harsha A; Singh, Rakesh Kumar; Singh, Sunil Kumar; Jaiswal, Manish; Jaiswal, Somil; Srivastava, Chhitij; Ojha, Balkrishna; Chandra, Anil; Chhabra, Satnam

    Intradural migration of disc (IDMD) is a rare clinical entity accounting for 0.27-0.33% of all herniated disc diseases. Flimsy or dense adhesion between the ventral dural surface and the opposing posterior longitudinal ligament (PLL) is the principal pathology for intradural migrated disc. The most commonly affected lumbar segments are L4-5 (55%), L3-4 (16%), L5-S1 (10%) and less commonly L2L3 and L1L2. No imaging feature is characteristic and the management protocol of durotomy via an endoscopic method is unclear. An L5S1 disc disease was operated by endoscopic method. Difficulty in separating the dural sac from PLL, dense adhesions prompting sharp dissection at this location and a calcified disc are the earliest evidence of intradural migration. MRI features of an intradural location are loss of continuity of posterior longitudinal ligament, beak-like appearance also known as "Hawk-beak sign", peripheral enhancement around an intradural disc, fluid-filled intradural cyst. Magnification either by Microscope or Endoscope is of importance when dissecting the intradural disc so as to avoid the nerve root injury. Liberal use of fibrin glue and augmentation with muscle patch was performed. Ambulated by 48h and discharged by 5th day. Two and 9 months follow up showed no evidence of pseudomeningocoele. Autologous muscle patch with fibrin glue for dural rent closure is a simple and effective method which can be performed by endoscopic or minimally invasive approaches. Suturing the dura, being a tedious and cumbersome procedure can be avoided. Copyright © 2016. Published by Elsevier Urban & Partner Sp. z o.o.

  6. Localization and Treatment of Unruptured Paraclinoid Aneurysms: A Proton Density MRI-based Study.

    Science.gov (United States)

    Yoon, Seon Jin; Shin, Na-Young; Lee, Jae Whan; Huh, Seung Kon; Park, Keun Young

    2015-09-01

    The purpose of this study was to evaluate the usefulness of proton density magnetic resonance (PD MR) imaging for localization of paraclinoid internal carotid artery aneurysms. From April 2014 to April 2015, 76 unruptured paraclinoid aneurysms in 66 patients were evaluated using PD MR and angiography (CT/MR angiography or digital subtraction angiography). The locations (extradural, transdural, intradural) in relation to the distal dural ring (DDR) and projection (superior, inferior/posterior, medial, lateral) of the aneurysms were assessed and compared. The most common location of paraclinoid aneurysms was extradural (n = 48, 63.2%), followed by intradural (n = 18, 23.7%), and transdural (n = 10, 13.2%). In the medial projection group (n = 49, 64.5%), 31 were extradural (63.3%), 5 were transdural (10.2%), and 13 were intradural (26.5%). In the inferior/posterior projection group (n = 19, 25.0%), there were 14 extradural (73.7%), 4 transdural (21.0%), and 1 intradural (5.3%). In the superior (n = 4, 5.3%)/lateral (n = 4, 5.3%) projection groups, there were 0/3 extradural (0/75.0%), 1/0 transdural (25.0/0%), and 3/1 intradural (75.0/25.0%). PD MR showed sufficient contrast difference to distinguish paraclinoid aneurysms from surrounding dural structures.

  7. Coexistence of neurofibroma and meningioma at exactly the same level of the cervical spine

    Directory of Open Access Journals (Sweden)

    Kai-Yuan Chen

    2014-11-01

    Full Text Available We report a case of the coexistence of different spinal tumors at the same level of the cervical spine, without neurofibromatosis (NF, which was successfully treated with surgery. A 72-year-old female presented with right upper-limb clumsiness and weakness. Magnetic resonance imaging revealed an intradural, extramedullary tumor mass at the right C3–4 level with extradural extension into the intervertebral foramen. The extradural tumor was removed, and the pathology showed neurofibroma. After incision of the dura, the intradural tumor was removed, and was identified as meningioma in the pathological report. The patient did not meet the criteria of NF. Coexistence of neurofibroma and meningioma at exactly the same level of the spine without NF is extremely rare. Exploration of the intradural space may be necessary after resection of an extradural tumor if the surgical finding does not correlate well with the preoperative images.

  8. Treatment of empty sella associated with visual impairment: a systematic review of chiasmapexy techniques.

    Science.gov (United States)

    Barzaghi, Lina Raffaella; Donofrio, Carmine Antonio; Panni, Pietro; Losa, Marco; Mortini, Pietro

    2018-02-01

    Chiasmapexy is a poorly described surgical procedure adopted to correct the downward displacement of suprasellar visual system (SVS) into an empty sella (ES) causing visual worsening. The aim of our study is to define the indications for extradural and intradural chiasmapexy. A systematic literature review has been performed on MEDLINE database (US National Library of Medicine), including only articles that depicted cases of surgically treated patients affected by ES and progressive delayed visual worsening. Moreover, we have reported three cases of secondary ES syndrome (SESS) with visual worsening treated in our Department with transsphenoidal (TS) microsurgical intradural approach. Finally, we have compared the results of extradural and intradural chiasmapexy described in literature. The etiology of visual impairment is different in primary and secondary ESS. In primary ESS (PESS) the only predisposing factor is a dehiscence of diaphragma sellae, and the anatomical distortion caused by displacement of optic chiasm or traction of pituitary stalk and infundibulum may determine a direct injury of neural fibers and ischemic damage of SVS. In PESS the mechanical elevation of SVS performed through extradural approach is sufficient to resolve the main pathologic mechanism. In SESS, arachnoidal adhesions play an important role in addition to downward herniation of SVS. Consequently, the surgical technique should provide elevation of SVS combined to intradural release of scar tissue and arachnoidal adhesions. In treatment of SESS, the intradural approaches result to be more effective, guaranteeing the best visual outcomes with the lowest complications rates. The intradural chiasmapexy is indicated in treatment of SESS, instead the extradural approaches are suggested for surgical management of PESS.

  9. Proton therapy for tumors of the skull base

    Energy Technology Data Exchange (ETDEWEB)

    Munzenrider, J.E.; Liebsch, N.J. [Dept. of Radiation Oncology, Harvard Univ. Medical School, Boston, MA (United States)

    1999-06-01

    Charged particle beams are ideal for treating skull base and cervical spine tumors: dose can be focused in the target, while achieving significant sparing of the brain, brain stem, cervical cord, and optic nerves and chiasm. For skull base tumors, 10-year local control rates with combined proton-photon therapy are highest for chondrosarcomas, intermediate for male chordomas, and lowest for female chordomas (94%, 65%, and 42%, respectively). For cervical spine tumors, 10-year local control rates are not significantly different for chordomas and chondrosarcomas (54% and 48%, respectively), nor is there any difference in local control between males and females. Observed treatment-related morbidity has been judged acceptable, in view of the major morbidity and mortality which accompany uncontrolled tumor growth. (orig.)

  10. Transradicular lumbar disc herniation: An extreme variant of intraradicular disc herniation.

    Science.gov (United States)

    Kasliwal, Manish K; Shimer, Adam L

    2015-01-01

    Intradural or intraradicular lumbar disc herniation (IDH) is a relatively rare condition often diagnosed intraoperatively. We encountered an extreme variant of IDH - a transradicular herniation as the disc material extruded through the lumbar nerve root through a split essentially transecting the nerve root. While failure to recognize intradural and intraradicular disc herniation can lead to failed back surgery, the variant described in the present case could lead to iatrogenic injury and complication if not recognized. A unique case of transradicular lumbar disc herniation in a 25-year-old patient is presented with the depiction of intraoperative images supplementing the text.

  11. Transradicular lumbar disc herniation: An extreme variant of intraradicular disc herniation

    Directory of Open Access Journals (Sweden)

    Manish K Kasliwal

    2015-01-01

    Full Text Available Intradural or intraradicular lumbar disc herniation (IDH is a relatively rare condition often diagnosed intraoperatively. We encountered an extreme variant of IDH - a transradicular herniation as the disc material extruded through the lumbar nerve root through a split essentially transecting the nerve root. While failure to recognize intradural and intraradicular disc herniation can lead to failed back surgery, the variant described in the present case could lead to iatrogenic injury and complication if not recognized. A unique case of transradicular lumbar disc herniation in a 25-year-old patient is presented with the depiction of intraoperative images supplementing the text.

  12. Updates in the treatment of bone cancer.

    Science.gov (United States)

    Biermann, J Sybil

    2013-05-01

    Although extremely rare, primary bone cancers are often curable with proper treatment. Effective management of primary bone tumors hinges on the involvement of a multidisciplinary team of physicians with expertise in this area, both in the realms of diagnosis and treatment. In her presentation at the NCCN 18th Annual Conference, Dr. J. Sybil Biermann reviewed the changes to the 2013 NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for bone cancer, featuring the introduction of new sections on giant cell tumor of bone (GCTB) and chordoma. The benefits of denosumab for the benign GCTB and the unique challenges associated with the malignant chordoma are also explored.

  13. Coccydynia due to a benign notochordal cell tumor.

    Science.gov (United States)

    Haasper, Carl; Länger, Florian; Rosenthal, Herbert; Knobloch, Karsten; Mössinger, Eckart; Krettek, Christian; Bastian, Leonhard

    2007-06-15

    Case report. To present a rare case of a notochordal cell tumor. We report on a 27-year-old female patient with pain at the lower back and muscle cramps in the area of the right hip. Image studies demonstrated a cystic lesion of the coccyx. As clinical symptoms became chronic and were resistant to conservative treatment, a resection of the coccyx was performed. Histology revealed an intraosseous benign notochordal cell tumor. This tumor represents a recently described notochordal cell proliferation biologically distinct from chordomas. Overdiagnosis of these notochordal cell proliferations as chordomas may occur if clinicians and pathologists are unfamiliar with the spectrum of notochordal proliferations.

  14. African Journal of Neurological Sciences - 2009 Vol. 28 No 1

    African Journals Online (AJOL)

    ADELOLA ADOLOYE. Neurosurgery in Africa. 1st ed. Ibadan: Ibadan University Press 1989:386. 2. ANDREW T PARSA, JANET LEE, IAN F PARNEY, PHILIP WEINSTEIN, PAUL C. MC CORMICK,. CHRISTOPHER AMES. Spinal cord and intradural-extraparenchymal spinal tumors: current best care practices and strategies.

  15. Spinal metastasis of occult lung carcinoma causing cauda equina syndrome.

    Science.gov (United States)

    Kotil, Kadir; Kilinc, Bekir Mahmut; Bilge, Turgay

    2007-04-01

    Cauda equina syndrome (CES) may be caused by tumor, herniated disc, trauma and spinal infections. However, CES due to occult lung cancer has not been reported in the literature. A 50-year-old man presented with a subacute CES caused by an intradural metastasis of an adenocarcinoma of the lung to the lumbosacral cauda fibers. His lumbosacral magnetic resonance imaging (MRI), showed a well-demarcated, intradural extramedullary mass lesion resembling a neurinoma at the L4/5 level. The patient underwent an L4-L5 laminectomy. The operative findings were also suggestive of neurinoma with involvement of three nerve roots, and a well-demarcated tumor without infiltration into the subarachnoid space. Although the findings of the operation were suggestive of neurinoma, final pathological diagnosis revealed metastatic carcinoma. Immunohistochemistry revealed clear cell adenocarcinoma metastasis. Chest X-ray and high resolution contrasted pulmonary computed tomography were normal. Positron emission tomography (PET) showed a lung mass, at the left apex. The patient was treated with chemotherapy and post-operative spinal radiotherapy was also performed. The CES resolved after the operation and the patient was followed up for 2 years with no recurrence. MRI of intradural cauda equina metastasis may be similar to that of intradural nerve sheath tumor. Surgery and postoperative radiotherapy may be effective for the treatment of CES due to lung carcinoma. Definitive diagnosis is by histopathological examination with immunohistochemistry. If the primary cancer cannot be detected by conventional radiological techniques, PET may be helpful.

  16. Necrotizing leptomeningeal vasculitis associated with a compressive meningioma in a cat: a rare paraneoplastic syndrome

    Directory of Open Access Journals (Sweden)

    Les J. Gabor

    2012-02-01

    Full Text Available A 17 year old cat with a compressive meningioma was found to have an intradural, severe necrotizing vasculitis, spatially un associated with the neoplasm. Paraneoplastic vasculitis has been reported in two cases in the human literature associated with meningiomas. This is the first report of such an association in a domestic species.

  17. Intramedullary cavernous haemangioma of spinal cord: A case ...

    African Journals Online (AJOL)

    Thoracic myelography done showed bilateral symmetrical funnelling of the contrast at the level of T5 with widening of the spinal cord, which are typical characteristics of an intramedullary mass of spinal cord. T2-T6 Laminectomy was done. Near total excision of a 4 by 2.5 cm intradural, intramedulary bluish black, necrotic, ...

  18. Cervicothoracic Arachnoid Cyst Causing Cervical Myelopathy: A Case Report

    Directory of Open Access Journals (Sweden)

    Zahir Kizilay

    2015-03-01

    Full Text Available Several types of intraspinal cyst develop within the spinal canal from the craniovertebral junction to the sacrum. These lesions occur in both children and adults. Arachnoid cysts are one of them and are more frequent in the paediatric population, being a relatively uncommon lesion in adults. The arachnoid cyst may be located intradurally or extradurally. The intradural type may be congenital or from spinal trauma, infection or spondylosis. Although intradural arachnoid cysts are often asymptomatic, they may give early symptoms when they exist with synchronous pathologies constricting the spinal canal gradually as in cervical spondylosis.  In this report, a 60-year-old man with an arachnoid cyst of the cervicothoracic spine is presented. His cyst remained undiagnosed because of the nonspecific nature of the symptoms. It was only when he developed right hemiparesis that a posterior fluid collection compressing the spinal cord was found in Magnetic resonance imaginig. An intradural extramedullary cyst was removed with successful surgery and cord compression and symptoms were reversed. We discuss radiological diagnosis and surgical treatment of an arachnoid cyst in this report.

  19. Transformed chest chardomas in malignant fibrous histiocytorme: presentation of case and reviewing of literature

    International Nuclear Information System (INIS)

    Capelastegui, A.; Mateos, B.; Astigarraga, E.; Pastor, A.; Pomposo, I.; Egurbide, M.V.

    1994-01-01

    Chest chordomas are rare neoplasms, and their transformation into malignant fibrous histiocytoma (MFH) is even more exceptional. We present a new case, including magnetic resonance (MR) images. The literature on the subject is reviewed, focussing especially on the dorsal location of these neoplasms and their possible malignant transformation, as well as the role of MR in the assessment of these lesions. (Author)

  20. Brachyury Essential for Notochord Cell Fate, Not Proliferation or EMT | Center for Cancer Research

    Science.gov (United States)

    The Brachyury or T gene encodes a transcription factor that is essential for body axis elongation during embryonic development. T is also highly expressed in chordomas, rare sarcomas derived from notochord cells, and a number of additional tumor types, including lung, prostate, and colon cancers. 

  1. Chondrosarcoma of the temporal bone: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Man Soo; Lee, Sang Youl; Chung, Jae Gul; Lee, Deok Hee; Jung, Seung Mun; Ryu, Dae Sik [Kang Nung Hospital, Ulsan Univ. Kangnung (Korea, Republic of)

    2001-07-01

    Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with chordoma, glomus jugulare tumor and meningioma, among other conditions, and due to its anatomic location, cranial nerve palsy is frequently observed. We report a case involving a 50-year-old woman with chondrosarcoma of the temporal bone.

  2. Extradural spinal meningioma: Revisiting a rare entity

    Directory of Open Access Journals (Sweden)

    Guruprasad Bettaswamy

    2016-01-01

    Full Text Available Spinal meningiomas are mostly intradural in location although at times these are associated with some extradural extensions. Purely extradural spinal meningiomas (EDSMs are however, extremely rare and when present, may cause diagnostic dilemma preoperatively. Only seven cases of pure EDSM have been reported till date. In this paper, we describe two cases of EDSM affecting the cervical spine and present their clinical profiles, radiological findings, operative management, and follow-up data, along with a review of the literature.

  3. Evaluation of the post-operative lumbar spine with MR imaging. The role of contrast enhancement and thickening in nerve roots

    Energy Technology Data Exchange (ETDEWEB)

    Grane, P.; Lindqvist, M. [Karolinska Hospital, Stockholm (Sweden). Dept. of Neuroradiology

    1997-11-01

    Purpose: Two new signs of lumbar nerve-root affection have been reported in recent years on the basis of MR examinations, namely: thickening in nerve roots; and contrast enhancement in nerve roots. The aim of this study was to assess contrast enhancement in nerve roots in a standardised way, and to evaluate the clinical significance of contrast enhancement and of nerve-root thickening in the symptomatic post-operative lumbar spine. Material and Methods: A total of 121 patients (who had previously been operated on for lumbar disc herniation) underwent 152 MR examinations, mainly on a 1.5 T system. Focal nerve-root enhancement was identified by visual assessment. Intradural enhancement was also quantified by pixel measurements that compared the affected nerve roots before and after contrast administration. Non-affected nerve roots were used as reference. Results: Enhanced nerve roots in the dural sac increased at least 40-50% in signal intensity after contrast administration compared to pre-contrast images and also compared to non-affected nerve roots. Intradural nerve-root enhancement was seen in 10% of the patients and focal enhancement in the root sleeve was seen in a further 26%. Nerve-root thickening was seen in 30%. Good correlation with clinical symptoms was found in 59% of the patients with intradural enhancement, in 84% with focal enhancement, and in 86% with nerve-root thickening. The combination of thickening and enhancement in the nerve root correlated with symptoms in 86% of the patients. Conclusion: Nerve-root enhancement (whether focal or intradural) and thickening in the nerve root are significant MR findings in the post-operative lumbar spine. In conbination with disc herniation or nerve-root displacement, these two signs may strengthen the indication for repeat surgery. However, root enhancement within 6 months of previous surgery may be a normal post-operative finding. (orig.).

  4. Two spinal arachnoid cysts

    Energy Technology Data Exchange (ETDEWEB)

    Puijlaert, J.B.C.M.; Vielvoye, G.J.; Dulken, H. van

    1985-05-01

    Two cases of spinal arachnoid cysts are reported. One is extradurally located, the other intradurally. The first is only documented with myelography, the second also by subsequent CT scanning. Some clinical and diagnostic aspects of the lesion are discussed. The aim of this report is to add two new cases to the literature and to emphasize the role of high-resolution CT scanning in the diagnosis of these lesions.

  5. Spinal infections

    International Nuclear Information System (INIS)

    Tali, E. Turgut; Gueltekin, Serap

    2005-01-01

    Spinal infections have an increasing prevalence among the general population. Definitive diagnosis based solely on clinical grounds is usually not possible and radiological imaging is used in almost all patients. The primary aim of the authors is to present an overview of spinal infections located in epidural, intradural and intramedullary compartments and to provide diagnostic clues regarding different imaging modalities, particularly MRI, to the practicing physicians and radiologists. (orig.)

  6. Intraspinal schwannoma and neurogenic bladder.

    Science.gov (United States)

    Yang, K S; Ho, C S; Tai, P A; Kung, W M

    2018-04-01

    Most lumbar intradural schwannomas present initially as radiculopathies with sensory disturbances. However, neurogenic bladder dysfunction may be one of the earliest manifestations and can cause long-term disability. We present the case of a patient with a L3-4 schwannoma (newly diagnosed owing to recurrent urinary retention and urinary tract infection) who finally underwent surgical resection. Improvement of bladder sensation was documented by urodynamic study and the patient was subsequently weaned off her Foley catheter with satisfactory outcome.

  7. Evaluation of spinal cord vessels using multi-slice CT angiography

    International Nuclear Information System (INIS)

    Chen Shuang; Zhu Ruijiang; Feng Xiaoyuan

    2006-01-01

    Objective: To evaluate the value of Multi-slice spiral CT angiography for spinal cord vessels. Methods: 11 adult subjects with suspected of myelopathy were performed with Multi-slice spiral CT angiography, An iodine contrast agent was injected at 3.5 ml/s, for total 100 ml. The parameters were axial 16 slice mode, 0.625 mm slice thickness, 0.8 s rotation, delay time depending on smartprep(15-25 s), multi-phase scan. The coronal and sagittal MPR and SSD were generated on a workstation compared with spinal digital subtraction angiography (DSA) to analyze normal or abnormal spinal cord vessels. Results: Normal findings at spinal CTA and digital subtraction angiography in six adult normal subjects and spinal cord vascular malformations (1 intradural extramedullary AVF, 4 dural AVFs) in five cases, Recognizable intradural vessels corresponding to anterior median (midline) veins and/or anterior spinal arteries were show in six adult normal subjects. Abnormal intradural vessels were detected in all five spinal cord vascular malformation with CT angiography, in comparison with digital subtraction angiography these vessels were primarily enlarged veins of the coronal venous plexus on the cord surface, radiculomedullary-dural arteries could not be clearly shown in four dural AVF, only one anterior spinal artery was detected in one patient with intradural medullary AVF, which direct shunt between anterior spinal artery and perimedullary vein with tortuous draining vessel. Conclusion: Multi-slice CT angiography is able to visualize the normal or abnormal spinal cord vessels. It could be used as a noninvasive method to screen the spinal cord vascular disease. (authors)

  8. The radiological and histopathological differential diagnosis of chordoid neoplasms in skull base

    Directory of Open Access Journals (Sweden)

    PAN Bin-cai

    2013-07-01

    Full Text Available Background Chordoid neoplasms refer to tumors appearing to have histological features of embryonic notochord, which is characterized by cords and lobules of neoplastic cells arranged within myxoid matrix. Because of radiological and histological similarities with myxoid matrix and overlapping immunohistochemical profile, chordoma, chordoid meningioma, chordoid glioma, and rare extraskeletal myxoid chondrosarcoma enter in the radiological and histological differential diagnosis at the site of skull base. However, there is always a great challenge for histopathologists to make an accurate diagnosis when encountering a chordoid neoplasm within or near the central nervous system. The aim of this study is to investigate and summarize the radiological, histological features and immunohistochemical profiles of chordoid neoplasms in skull base, and to find a judicious panel of immunostains to unquestionably help in diagnostically challenging cases. Methods A total of 23 cases of chordoid neoplasms in skull base, including 10 chordomas, 5 chordoid meningiomas, 3 chordoid gliomas and 5 extraskeletal myxoid chondrosarcomas, were collected from the First Affiliated Hospital, Sun Yat-sen University and Guangdong Tongjiang Hospital. MRI examination was performed on the patients before surgical treatment. Microscopical examination and immunohistochemical staining study using vimentin (Vim, pan-cytokeratin (PCK, epithelial membrane antigen (EMA, S?100 protein (S-100, glial fibrillary acidic protein (GFAP, D2-40, Galectin-3, CD3, CD20, Ki-67 were performed on the samples of cases. The clinicopathological data of the patients was also analyzed retrospectively. Results Most of chordomas were localized in the clivus with heterogeneous hyperintensity on T2WI scanning. The breakage of clivus was observed in most cases. Histologically, the tumor cells of chordoma exhibited bland nuclear features and some contained abundant vacuolated cytoplasm (the so

  9. Metastase de carcinoma comprometendo a cauda equina Metastatic carcinoma of the cauda equina: a case report

    Directory of Open Access Journals (Sweden)

    Lígia M. B. Coutinho

    1976-09-01

    Full Text Available É relatado um caso de paciente, de 60 anos, que apresentou tumor nos segmentos apical e posterior direitos, cujo diagnóstico histopatológico foi de carcinoma indiferenciado. O paciente foi submetido à cobaltoterapia, tendo melhorado por três meses, quando foi novamente hospitalizado por dor lombar. A mielografia com lipiodol mostrou processo expansivo intrarraqueano. Mediante cirurgia foi encontrado tumor intra-dural, englobando raízes nervosas. O diagnóstico microscópico foi de carcinoma indiferenciado infiltrando os espaços epi e peri-neurais.The case of a 60 year-old man who had an indifferenciated carcinoma in the lung is reported. He had recieved cobaltotherapy and had improved. After 3 months a lumbar pain had begun and the patient was hospitalized. A myelography with lipiodol demonstrated an intra-dural mass. At operation a big intra-dural tumor including the cauda equina was found. The microscopic examination revealed an undifferenciated carcinoma, that infiltrated the epi and peri-neural space.

  10. Novel dural incision and closure procedure for preventing postoperative cerebrospinal fluid leakage during the surgical removal of dumbbell-shaped spinal tumors: technical note.

    Science.gov (United States)

    Ito, Kiyoshi; Aoyama, Tatsuro; Nakamura, Takuya; Hanaoka, Yoshiki; Horiuchi, Tetsuyoshi; Hongo, Kazuhiro

    2016-11-01

    The authors report on a new method for removing dumbbell-shaped spinal tumors that avoids the risk of postoperative cerebrospinal fluid (CSF) leakage. Adequate visualization of the intra- and extradural components of the tumor is achieved with the use of separate dural incisions. First, the dura mater is opened along the dural theca to provide adequate visualization of the intradural portion of the mass; then, a second incision is made along the nerve root to remove the extradural component. Meticulous suturing is essential in intradural lesion cases; however, the dura mater is usually thin and fragile in such cases. During suturing with a needle and thread, the dura mater can become lacerated proximal to the needle holes and result in CSF leakage. In the authors' technique, instead of using a needle and thread, nonpenetrating vascular clips were used to close the dural incisions. When operating on dumbbell-shaped spinal tumors, the authors found that the "separate-dural-incision method" was preferable to the conventional T-shaped dural incision method because no dural defects occurred after the intradural procedure and meticulous dural closure with vascular clips was achieved. The authors conclude that the novel separate-dural-incision method for removing dumbbell-shaped tumors and the use of nonpenetrating vascular clips permits reliable dural closure, prevents postoperative CSF leakage, and promises good postoperative clinical results.

  11. Isolated unilateral hypoglossal nerve palsy secondary to an atlantooccipital joint juxtafacet synovial cyst.

    Science.gov (United States)

    Elhammady, Mohamed Samy; Farhat, Hamad; Aziz-Sultan, Mohammad Ali; Morcos, Jacques J

    2009-03-01

    Juxtafacet cysts of the atlantooccipital joint that present with isolated hypoglossal nerve palsy are rare and may mimic more common pathological entities. The authors report on the third such case in the literature and discuss the differential diagnosis, imaging hallmarks, preoperative recognition, and surgical management of this lesion, and provide a review of the literature. The authors discuss their experience with the treatment of a 67-year-old woman who presented with an isolated hypoglossal nerve palsy caused by a nonenhancing cystic septated lesion abutting the lateral medulla just medial to the left hypoglossal canal. The lesion was presumed to be a necrotic hypoglossal schwannoma or epidermoid tumor. Intradural surgical exploration failed to demonstrate an intradural lesion, but confirmed the presence of an extradural mass caudal to the hypoglossal nerve. Extradural exploration revealed a synovial cyst of the atlantooccipital joint, which was then resected. Postoperatively, the patient developed worsening dysphagia and hoarseness. Failure to recognize this rare entity preoperatively resulted in unnecessary intradural exploration and cranial nerve morbidity. In retrospect, the preoperative diagnosis of this lesion was suggested by lack of central enhancement, absence of dumbbell formation and the presence of erosive synovial changes. Regardless, the extreme rarity of this lesion at this location will always make its recognition challenging.

  12. A Novel Type of Ureteral Stents in the Treatment of a Bilateral Iatrogenic Transaction of the Ureters

    Directory of Open Access Journals (Sweden)

    Ernesto Mazza

    2013-01-01

    Full Text Available This report illustrates the case of a patient who suffered an iatrogenic complete injury of both ureters after a complex surgical procedure to remove a large sacral chordoma. Ureteral recanalization was achieved with two removable, autoexpandable, and polytetrafluoroethylene covered nitinol stents. To our knowledge, we describe the first application of this type of stents to treat a bilateral ureteral transection. Despite the bad general conditions of the patient, the ureteral stents successfully restored and maintained the bilateral ureteral continuity.

  13. Case report: Greater meningeal inflammation in lumbar than in ventricular region in human bacterial meningitis

    OpenAIRE

    Naija, Walid; Matéo, Joaquim; Raskine, Laurent; Timsit, Jean-François; Lukascewicz, Anne-Claire; George, Bernard; Payen, Didier; Mebazaa, Alexandre

    2004-01-01

    Differences in the composition of ventricular and lumbar cerebrospinal fluid (CSF) based on single pairs of samples have previously been described. We describe a patient that developed post-surgical recurrent meningitis monitored by daily biochemical and bacteriological CSF analysis, simultaneously withdrawn from lumbar space and ventricles. A 20-year-old Caucasian man was admitted to the ICU after a resection of a chordoma that extended from the sphenoidal sinus to the anterior face of C2. C...

  14. Bone tumors of the spine and sacral bone

    International Nuclear Information System (INIS)

    Freyschmidt, Juergen

    2010-01-01

    Bone tumors and tumorlike lesions of the spine are rare entities and may harbour diagnostic problems. In this article we discuss the epidemiology, topographic aspects, clinical and radiologic features as well as the diagnostic management of vertebral bone tumors. Entities that should be more familiar to the radiologist (i.e. osteoid osteoma, osteoblastoma, hemangioma, giant cell tumor, chordoma, aneurysmal bone cyst) are considered in more detail. (orig.)

  15. Bone tumors of the spine and sacral bone; Primaere Tumoren der Wirbelsaeule und des Sakrums

    Energy Technology Data Exchange (ETDEWEB)

    Freyschmidt, Juergen [Klinikum Bremen-Mitte gGmbH, Bremen (Germany). Beratungsstelle und Referenzzentrum fuer Osteoradiologie

    2010-12-15

    Bone tumors and tumorlike lesions of the spine are rare entities and may harbour diagnostic problems. In this article we discuss the epidemiology, topographic aspects, clinical and radiologic features as well as the diagnostic management of vertebral bone tumors. Entities that should be more familiar to the radiologist (i.e. osteoid osteoma, osteoblastoma, hemangioma, giant cell tumor, chordoma, aneurysmal bone cyst) are considered in more detail. (orig.)

  16. [Intracranial collision tumor--A case report (author's transl)].

    Science.gov (United States)

    Fukaya, T; Yoshida, J; Banno, T; Kageyama, N; Ito, M

    1976-06-01

    A 21-year-old man with nasopharyngeal tumor was first admitted to the Nagoya University Hospital on April 15, 1972. He had difficulty in speaking and swallowing, and developed double vision prior to admission. A soft and yellow tumor was found in the nasopharynx and revealed typical features of chordoma. The patient underwent Co60 irradiation after the operation. On January 25, 1973, the patient developed double vision of severe degree. Microscopic examination of the specimen which was obtained at the time of the second operation in February 9, 1973, disclosed a coexistence (collision) of chordoma and hemangioblastoma. The two different tumors were situated in juxtaposition on histological examination. Co60 irradiation was added during his second hospitalization. Three months after the second operation, he developed symptoms of meningitis and was hospitalized for the third time on June 3, 1973, at which time the tumor tissue extended through the right frontal and middle fossa. The third operation was done with frontal craniotomy and tumor was partially removed. The histological diagnosis was hemangioblastoma. Postoperatively the patient went downhill and died on September 19, 1973. The report of a collision tumor of intracranial chordoma and hemangioblastoma is not found in the previous literature. There have been many theories as to the origin of collision tumor. Some investigators have proposed that the existence of hyperplastic blood vessels within the glioblastoma is responsible for the collision tumor of sarcoma and glioblastoma. Since the advent of radiotherapy, several examples of sarcoma have been discovered at postmortem examination in patient irradiated for treatment of cerebral neoplasm, both gliogeneous and nongliogenous, suggesting a possible relationship between the tumor and the radiation therapy. In our case, the chordoma showed neither hyperplastic blood vessels nor malignant pattern on histological examination. It was suspected that post

  17. Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma

    Science.gov (United States)

    2017-12-11

    Adult Rhabdomyosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Previously Untreated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  18. A dose-escalating phase I of imatinib mesylate with fixed dose of metronomic cyclophosphamide in targeted olid tumours

    Science.gov (United States)

    Adenis, A; Ray-Coquard, I; Italiano, A; Chauzit, E; Bui-Nguyen, B; Blay, J-Y; Tresch-Bruneel, E; Fournier, C; Clisant, S; Amela, E Y; Cassier, P A; Molimard, M; Penel, N

    2013-01-01

    Background: Preclinical findings suggest that imatinib mesylate (IM) and metronomic cyclophosphamide (MC) combination provides synergistic antiangiogenic activity on both pericytes and endothelial cells. Methods: We have designed a 3+3 dose-escalating phase I trial with a fixed dose of MC (50 mg two times daily) plus IM (400 mg per day; 300 and 400 mg two times daily). Enrolled patients had IM- and sutininib-refractory advanced gastrointestinal stromal tumours (GIST) (n=17), chordoma (n=7) and mucosal melanoma (n=2). Dose-limiting toxicities were monitored for the first 6 weeks. Progression-free survival (PFS) and response assessment are based on RECIST 1.0 guidelines. Pharmacokinetics of IM were measured before and after exposure to MC. Results: No dose-limiting toxicity was observed. Fourteen patients of the expanded cohort received 400 mg two times daily of IM with MC. Apart from a case of possibly related acute leukaemia occurring after 4 years of treatment, we did not see unexpected toxicity. No drug–drug pharmacokinetic interaction was observed. There was no objective response. We have observed long-lasting stable disease in chordoma patients (median PFS=10.2 months; range, 4.2–18+) and short-term stable disease in heavily GIST pretreated patients (median PFS=2.3 months; range, 2.1–6.6). Conclusion: This combination is feasible and may warrant further exploration in refractory GIST or chordoma patients. PMID:24149182

  19. A dose-escalating phase I of imatinib mesylate with fixed dose of metronomic cyclophosphamide in targeted solid tumours.

    Science.gov (United States)

    Adenis, A; Ray-Coquard, I; Italiano, A; Chauzit, E; Bui-Nguyen, B; Blay, J-Y; Tresch-Bruneel, E; Fournier, C; Clisant, S; Amela, E Y; Cassier, P A; Molimard, M; Penel, N

    2013-11-12

    Preclinical findings suggest that imatinib mesylate (IM) and metronomic cyclophosphamide (MC) combination provides synergistic antiangiogenic activity on both pericytes and endothelial cells. We have designed a 3+3 dose-escalating phase I trial with a fixed dose of MC (50 mg two times daily) plus IM (400 mg per day; 300 and 400 mg two times daily). Enrolled patients had IM- and sutininib-refractory advanced gastrointestinal stromal tumours (GIST) (n=17), chordoma (n=7) and mucosal melanoma (n=2). Dose-limiting toxicities were monitored for the first 6 weeks. Progression-free survival (PFS) and response assessment are based on RECIST 1.0 guidelines. Pharmacokinetics of IM were measured before and after exposure to MC. No dose-limiting toxicity was observed. Fourteen patients of the expanded cohort received 400 mg two times daily of IM with MC. Apart from a case of possibly related acute leukaemia occurring after 4 years of treatment, we did not see unexpected toxicity. No drug-drug pharmacokinetic interaction was observed. There was no objective response. We have observed long-lasting stable disease in chordoma patients (median PFS=10.2 months; range, 4.2-18+) and short-term stable disease in heavily GIST pretreated patients (median PFS=2.3 months; range, 2.1-6.6). This combination is feasible and may warrant further exploration in refractory GIST or chordoma patients.

  20. Anteriorly placed tumors to the conus: removal by interradicular window.

    Science.gov (United States)

    Ciappetta, P; Taurone, S; Spoletini, M; Artico, M

    2017-01-01

    Tumors anteriorly situated to the medullary conus are rarely encountered and represent a true surgical challenge. We examined the literature on this topic, concluding that there are no previous reports on alternative surgical techniques different to the traditional one. We report two cases of intradural extramedullary tumor operated on by a technique performed through a window opened between the spinal roots, which allows an easy, effective and useful resection. We describe a new operative technique which ensures a complete removal of these tumors and discuss clinical implications in the light of the available literature on this topic.

  1. Spinal cord arteriovenous shunts: from imaging to management

    International Nuclear Information System (INIS)

    Rodesch, G.; Lasjaunias, P.

    2003-01-01

    Spinal cord arteriovenous shunts (SCAVSs) are either fistulas or niduses that can be separated in four different groups according to their localization and relationship to the dura. Paraspinal AVSs are located outside the spine and are responsible for neurological symptoms because of cord compression by ertatic veins, venous congestion or arterial steal. Epidural shunts are located in the epidural space and drain in epidural veins with secondary intradural congestion. Dural shunts are embedded in the dura, produce a cord venous myelopathy after draining through veins that either pierce the dura far from a nerve root or accompany a nerve root. Intradural shunts affect the cord, the roots or the filum. Additionally, they can be classified according to their potential relationships with genetics, vascular biological features and angiogenesis into genetic hereditary lesions (hereditary hemorrhagic telangiectasia), genetic non-hereditary lesions (multiple lesions with metameric links) and single lesions (AVMs or micro AVFs). MRI and MRA are able to visualise SCAVS early after the onset of clinical symptoms. The type of shunt and its localization may remain difficult to be precise. Angiography remains the gold standard for analysis of the anatomical, morphological and architectural features necessary for therapeutic decisions in both paediatric and adult populations. In our series, embolisation is chosen in first intention whatever the type of shunt responsible for the clinical symptoms and glue is preferably used. In paraspinal, dural or epidural arteriovenous shunts, the goal of treatment should be complete closure of the shunt. A complete cure by embolization is rather easily achieved in paraspinal lesions. Failure of endovascular therapy in dural or epidural shunts must bring the patient to surgery. The prognosis of most intradural shunts seems better than previously thought, even after haemorrhage. In intradural spinal cord arteriovenous shunts, embolisation

  2. Repair of Temporal Bone Encephalocele following Canal Wall Down Mastoidectomy

    Directory of Open Access Journals (Sweden)

    Sarantis Blioskas

    2014-01-01

    Full Text Available We report a rare case of a temporal bone encephalocele after a canal wall down mastoidectomy performed to treat chronic otitis media with cholesteatoma. The patient was treated successfully via an intracranial approach. An enhanced layer-by-layer repair of the encephalocele and skull base deficit was achieved from intradurally to extradurally, using temporalis fascia, nasal septum cartilage, and artificial dural graft. After a 22-month follow-up period the patient remains symptom free and no recurrence is noted.

  3. Transjugular tubercle-preaccessory approach for ventrocaudally located PICA aneurysm.

    Science.gov (United States)

    Horiuchi, Tetsuyoshi; Nagm, Alhusain; Hasegawa, Takatoshi; Hongo, Kazuhiro

    2017-04-01

    Posterior inferior cerebellar artery (PICA) aneurysms are often located in the ventral craniocervical junction. Such deep-seated aneurysms are usually treated with coil embolization. However, recurred and/or very small PICA aneurysms are hardly treated with an endovascular approach. We present a modification of the transcondylar fossa approach with intradural and extradural removal of the jugular tubercle. An adequate preaccessory surgical corridor was created with this novel technique. The ventocaudal PICA aneurysm was clipped through the outside of the spinal accessory nerve without surgical manipulation of other lower cranial nerves. This approach is worthwhile to avoid dysphagia.

  4. Evaulación de la cefalea postpunción dural en población obstétrica: medidas profilácticas y eficacia del parche hemático epidural

    OpenAIRE

    Antuñano Unanua, Iranzu

    2016-01-01

    Objetivo:Este estudio analiza qué método profiláctico(catéter intradural versus nueva epidural) es más eficaz para evitar la cefalea postpunción dural (CPPD), así como la validez del parche hemático epidural (PHE). Material y métodos: Estudio observacional, descriptivo y retrospectivo. La población de estudio la constituyen 153 mujeres que solicitaron analgesia epidural de parto y que sufrieron una punción dural accidental (PDA) en el Complejo Hospitalario de Navarra desde 2003 hasta 2015....

  5. Iatrogenic intraspinal epidermoid tumor: Myelo-CT and MRI diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Visciani, A.; Balestrini, M.R.; Solero, C.L.; Savoiardo, M.

    1989-07-01

    An 11-year-old boy, treated for acute lymphatic leukemia at the age of 2 with intrathecal injections of Methotrexate, presented with a two year history of pain and signs of lumbo-sacral lesion. MRI, myelography and myelo-CT demonstrated an intradural L4-L5 epidermoid tumor which was removed. Iatrogenic implantation of epithelial cells at the age of two with lumbar punctures is most likely. Decline in incidence of lumbar iatrogenic epidermoid cysts, now an exceedingly rare event, is probably related to improved needles for lumbar punctures. (orig.).

  6. Iatrogenic intraspinal epidermoid tumor: Myelo-CT and MRI diagnosis

    International Nuclear Information System (INIS)

    Visciani, A.; Balestrini, M.R.; Solero, C.L.; Savoiardo, M.

    1989-01-01

    An 11-year-old boy, treated for acute lymphatic leukemia at the age of 2 with intrathecal injections of Methotrexate, presented with a two year history of pain and signs of lumbo-sacral lesion. MRI, myelography and myelo-CT demonstrated an intradural L4-L5 epidermoid tumor which was removed. Iatrogenic implantation of epithelial cells at the age of two with lumbar punctures is most likely. Decline in incidence of lumbar iatrogenic epidermoid cysts, now an exceedingly rare event, is probably related to improved needles for lumbar punctures. (orig.)

  7. Organising haematoma mimicking tumour on MRI following resection of acoustic neuroma

    International Nuclear Information System (INIS)

    Higgins, J.N.P.; Pigeon, C.N.; Moseley, I.F.

    1995-01-01

    We describe a 26-year-old woman in whom an enhancing, intradural extramedullary mass was found at the craniocervical junction on MRI 3 years after resection of a large acoustic neuroma. The radiological appearances suggested a new tumour, raising the possibility of neurofibromatosis 2, provoking a review of family members in an attempt to confirm the diagnosis, as well as further surgery. Histology of the lesion revealed an organising haematoma, with no evidence of malignancy. The imaging features can be explained by the process by which blood clot in the subarachnoid space is resorbed. Caution is advised in interpreting CT or MRI after neurosurgery. (orig.)

  8. Paraganglioma of the Cauda Equina Presenting with Erectile and Sphincter Dysfunction

    Directory of Open Access Journals (Sweden)

    Wiesław Marcol

    2009-06-01

    Full Text Available Paragangliomas of the cauda equina are rare neuroepithelial tumors, usually manifesting clinically as sciatica. Here, we report a case of cauda equina paraganglioma with an unusual course in a 43-year-old man. His main complaints were erectile and sphincter dysfunction. The low back pain was initially ascribed to accidental injury. Magnetic resonance imaging revealed intradural tumor at the L2/L3 level. The patient underwent gross tumor resection, and the diagnosis of paraganglioma was based on neuropathologic examination. The symptoms completely resolved after tumor resection.

  9. Markers aiding the diagnosis of chondroid tumors: an immunohistochemical study including osteonectin, bcl-2, cox-2, actin, calponin, D2-40 (podoplanin), mdm-2, CD117 (c-kit), and YKL-40

    Science.gov (United States)

    DAUGAARD, SØREN; CHRISTENSEN, LISE H; HØGDALL, ESTRID

    2009-01-01

    Chondroid tumors comprise a heterogenous group of benign to overt malignant neoplasms, which may be difficult to differentiate from one another by histological examination. A group of 43 such tumors was stained with nine relevant antibodies in an attempt to find consistent marker profile(s) for the different subgroups. Archival material from three extraskeletal myxoid chondrosarcomas, five chordomas, five chondromyxoid fibromas, five chondroblastomas and 25 chondrosarcomas was stained with antibodies against osteonectin, bcl-2, cox-2, actin, calponin, D2-40 (podoplanin), mdm-2, CD117 (c-kit) and YKL-40. All 25 chondrosarcomas showed a positive staining reaction for D2-40, none for actin and CD117, and a partial reactivity for bcl-2 (36%). Chondroblastomas (5/5) and chondromyxoid fibromas (2/5) were the only tumors with a positive reaction for actin, and all chondroblastomas (n=5) and extraskeletal myxoid chondrosarcomas (n=3) were positive for bcl-2. In contrast to all other tumors, two of three extraskeletal myxoid chondrosarcomas were also positive for CD17 and negative for osteonectin, cox-2, mdm-2 and actin. All five chordomas were negative for D2-40 and positive for mdm-2 and YKL-40. The diagnosis of chondrosarcoma may be aided by its positivity for D2-40 and YKL-40 and its lack of reactivity for actin and CD117. This should be seen in the light of no reaction for D2-40 in chordomas and a corresponding lack of reaction for osteonectin, cox-2, mdm-2 and actin in extraskeletal myxoid chondrosarcomas. A convincing immunoreactivity for calponin and/or actin in chondromyxoid fibromas and chondroblastomas may also be helpful in differentiating these tumors from chondrosarcomas. PMID:19594492

  10. Analysis of receptor tyrosine kinases (RTKs) and downstream pathways in chordomas†

    Science.gov (United States)

    Tamborini, Elena; Virdis, Emanuela; Negri, Tiziana; Orsenigo, Marta; Brich, Silvia; Conca, Elena; Gronchi, Alessandro; Stacchiotti, Silvia; Manenti, Giacomo; Casali, Paolo G.; Pierotti, Marco A.; Pilotti, Silvana

    2010-01-01

    We have previously demonstrated that chordomas express activated platelet-derived growth factor receptor (PDGFRB) and that treatment with imatinib, which is capable of switching off the activation of various receptor tyrosine kinases (RTKs) including PDGFRB, benefits a number of patients. The aim of this study was to identify the possible presence of other activated RTKs and their downstream signaling effectors. Cryopreserved material from 22 naïve sporadic chordomas was investigated for the presence of activated RTKs and their cognate ligands and downstream signaling effectors by means of human phospho-RTK antibody arrays, Western blotting, and molecular analysis; immunohistochemistry and fluorescence in situ hybridization were used to analyze the corresponding formalin-fixed and paraffin-embedded samples. We detected activated PDGFRB, FLT3, and colony stimulating factor 1 receptor (CSF1R) of the PDGFR family and highly phosphorylated EGFR, HER2/neu, and (to a lesser extent) HER4 of the EGFR family. The detection of PDGFRB/PDGFB confirmed our previous data. The presence of activated EGFR was paralleled by the finding of high levels of epidermal growth factor (EGF) and transforming growth factor α (TGFα) and PDGFB co-expression and PDGFRB co-immunoprecipitation. Of the downstream effectors, the PI3K/AKT and RAS/MAPK pathways were both activated, thus leading to the phosphorylation of mammalian target of rapamycin (mTOR) and 4E-BP1 among the regulators involved in translational control. Taken together, our results (i) provide a rationale for tailored treatments targeting upstream activated receptors, including the PDGFR and EGFR families; (ii) support the idea that a combination of upstream antagonists and mTOR inhibitors enhances the control of tumor growth; and (iii) indicate that the 4E-BP1/eIF4E pathway is a major regulator of protein synthesis in chordoma. PMID:20164240

  11. Imaging of painful solitary lesions of the sacrum

    International Nuclear Information System (INIS)

    Peh, W. C. G.; Koh, W. L.; Kwek, J. W.; Htoo, M. M.; Tan, P. H.

    2007-01-01

    Full text: In patients with sacral pain, the painful symptoms may be caused by a variety of bony and soft tissue lesions. Benign lesions include giant cell tumour, neurogenic tumour, insufficiency fracture, infection and giant bone island. Malignant lesions include primary bone tumours, Ewing sarcoma, plasmacytoma, lymphoma and chordoma. Soft tissue tumours adjacent to or involving the sacrum may cause painful symptoms. A multimodality approach to imaging is required for full assessment of these lesions. This pictorial essay describes a range of common solitary sacral lesions that may cause pain, with emphasis on imaging features

  12. Sixth cranial nerve palsy caused by gastric adenocarcinoma metastasis to the clivus.

    Science.gov (United States)

    Lee, Aleum; Chang, Kee-Hyun; Hong, Hyunsook; Kim, Heekyung

    2015-03-01

    Tumors of the clivus and metastases to the clivus are very rare. Metastasis involving the clivus has previously been described in only two case reports. In skull metastasis, the breast and prostate are the most common primary foci, while metastasis from gastric carcinoma is extremely rare. A review of the English literature revealed only one published case of clivus metastases from gastric adenocarcinoma. There is no literature thoroughly explaining the differential diagnosis between chordoma and metastasis. Here we report a rare case of metastasis to the clivus from a gastric adenocarcinoma in a 42-year-old female patient with sudden blurry vision, presenting as bilateral cranial nerve VI palsy.

  13. Imatinib in the treatment of solid tumours.

    Science.gov (United States)

    Duffaud, Florence; Le Cesne, Axel

    2009-01-01

    The extraordinary success of imatinib in gastrointestinal stromal tumors (GIST) represents a model for molecularly targeted therapy for other solid tumors. Research is currently going to identify the molecular basis of mechanisms of action and drug resistance. In this article, we review recent advances in the clinical management of patients with GISTs treated with imatinib, but also of patients with dermatofibrosarcoma protuberans, chordoma, aggressive fibromatosis, and some other common solid tumors treated with this drug. We reviewed the knowledge of the molecular mechanisms that are basic to imatinib effects in these tumors.

  14. A dose-escalating phase I of imatinib mesylate with fixed dose of metronomic cyclophosphamide in targeted olid tumours

    OpenAIRE

    Adenis, A; Ray-Coquard, I; Italiano, A; Chauzit, E; Bui-Nguyen, B; Blay, J-Y; Tresch-Bruneel, E; Fournier, C; Clisant, S; Amela, E Y; Cassier, P A; Molimard, M; Penel, N

    2013-01-01

    Background: Preclinical findings suggest that imatinib mesylate (IM) and metronomic cyclophosphamide (MC) combination provides synergistic antiangiogenic activity on both pericytes and endothelial cells. Methods: We have designed a 3+3 dose-escalating phase I trial with a fixed dose of MC (50 mg two times daily) plus IM (400 mg per day; 300 and 400 mg two times daily). Enrolled patients had IM- and sutininib-refractory advanced gastrointestinal stromal tumours (GIST) (n=17), chordoma (n=7) an...

  15. Acute onset of paraganglioma of filum terminale: A case report and surgical treatment.

    Science.gov (United States)

    Murrone, Domenico; Romanelli, Bruno; Vella, Giuseppe; Ierardi, Aldo

    2017-01-01

    Paragangliomas of filum terminale are rare benign tumors, arising from the adrenal medulla or extra-adrenal paraganglia. These lesions usually present with chronic back pain and radiculopathy and only two cases of acute neurological deficit have been reported in literature. A case with an acute paraplegia and cauda equina syndrome due to an hemorrhagic paraganglioma of the filum terminale is described. Magnetic resonance imaging showed an intradural tumor extending from L1 to L2 compressing the cauda equina, with an intralesional and intradural bleed. An emergent laminectomy with total removal of the tumor was performed allowing a post-operative partial sensory recovery. Histopathological examination diagnosed paraganglioma. Paragangliomas are solid, slow growing tumors arising from specialized neural crest cells, mostly occurring in the head and neck and rarely in cauda equina or filum terminale. MRI is gold standard radiological for diagnosis and follow-up of these lesions. They have no pathognomonic radiological and clinical features and are frequently misdiagnosed as other spinal lesions. No significant correlation was observed between the duration of symptoms and tumor dimension. Acute presentation is unusual and emergent surgical treatment is fondamental. The outcome is very good after complete excision and radiotherapical treatment is recommended after an incomplete resection. Early radiological assessment and timely surgery are mandatory to avoid progressive neurological deficits in case of acute clinical manifestation of paraganglioma of filum terminale. Copyright © 2017 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  16. CT and MRI findings of calcified spinal meningiomas: correlation with pathological findings

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ji Won; Kim, Hak Jin [Pusan National University Hospital, Department of Radiology, Busan (Korea); Pusan National University School of Medicine, Medical Research Institute, Busan (Korea); Lee, In Sook [Pusan National University Hospital, Department of Radiology, Busan (Korea); Pusan National University School of Medicine, Medical Research Institute, Busan (Korea); Pusan National University School of Medicine, Department of Radiology, Busan (Korea); Choi, Kyung-Un [Pusan National University School of Medicine, Medical Research Institute, Busan (Korea); Pusan National University Hospital, Department of Pathology, Busan (Korea); Lee, Young Hwan [Catholic University of Daegu School of Medicine, Department of Radiology, Daegu (Korea); Yi, Jae Hyuck [Kyungpook National University Hospital, Department of Radiology, Daegu (Korea); Song, Jong Woon [Inje University Pusan Paik Hospital, Department of Radiology, Busan (Korea); Suh, Kyung Jin [Dongguk University Gyungju Hospital, College of Medicine, Dongguk University, Department of Radiology, Gyungju (Korea)

    2010-04-15

    This study was designed to present characteristic CT and MR findings of calcified spinal meningiomas that correlate with pathological findings and to assess the efficacy of CT for the detection of calcifications within a mass in comparison to MRI. Between 1998 and 2009, 10 out of 11 patients who had pathologically confirmed psammomatous meningiomas showed gross calcifications on CT images and were included in this study. On CT scans of the 10 patients, the distribution pattern, morphology and number of calcifications within masses were evaluated. MRI was performed in seven patients and signal intensities of masses were assessed. The pathological results analyzed semi-quantitatively were compared with the density or the size of calcifications within a mass as seen on a CT scan. Seven of 10 masses were located at the thoracic spine level. Eight masses had intradural locations. The other two masses had extradural locations. Four masses were completely calcified based on standard radiographs and CT. Symptoms duration, the size of the mass and size or number of calcifications within a mass had no correlation. The location, size, and distribution pattern of calcifications within masses were variable. On MR images, signal intensity of calcified tumor varied on all imaging sequences. All the masses enhanced after injection of intravenous contrast material. A calcified meningioma should be first suggested when extradural or intradural masses located in the spine contain calcifications regardless of the size or pattern as depicted on CT, especially in the presence of enhancement as seen on MR images. (orig.)

  17. Radicular interdural lumbar disc herniation

    Science.gov (United States)

    Boulahroud, Omar; Elasri, Abad; Elmostarchid, Brahim; Boucetta, Mohammed

    2009-01-01

    Intraradicular lumbar disc herniation is a rare complication of disc disease that is generally diagnosed only during surgery. The mechanism for herniated disc penetration into the intradural space is not known with certainty, but adhesion between the radicular dura and the posterior longitudinal ligament was suggested as the most important condition. The authors report the first case of an intraradicular lumbar disc herniation without subdural penetration; the disc hernia was lodged between the two radicular dura layers. The patient, a 34-year-old soldier, was admitted with a 12-month history of low back pain and episodic left sciatica. Neurologic examination showed a positive straight leg raising test on the left side without sensory, motor or sphincter disturbances. Spinal CT scan and MRI exploration revealed a left posterolateral osteophyte formation at the L5–S1 level with an irregular large disc herniation, which migrated superiorly. An intradural extension was suspected. A left L5 hemilaminectomy and S1 foraminotomy were performed. The exploration revealed a large fragment of disc material located between the inner and outer layers of the left S1 radicular dura. The mass was extirpated without cerebrospinal fluid outflow. The postoperative course was uneventful. Radicular interdural lumbar disc herniation should be suspected when a swollen, hard and immobile nerve root is present intraoperatively. PMID:19888608

  18. Spinal tumors

    International Nuclear Information System (INIS)

    Goethem, J.W.M. van; Hauwe, L. van den; Oezsarlak, Oe.; Schepper, A.M.A. de; Parizel, P.M.

    2004-01-01

    Spinal tumors are uncommon lesions but may cause significant morbidity in terms of limb dysfunction. In establishing the differential diagnosis for a spinal lesion, location is the most important feature, but the clinical presentation and the patient's age and gender are also important. Magnetic resonance (MR) imaging plays a central role in the imaging of spinal tumors, easily allowing tumors to be classified as extradural, intradural-extramedullary or intramedullary, which is very useful in tumor characterization. In the evaluation of lesions of the osseous spine both computed tomography (CT) and MR are important. We describe the most common spinal tumors in detail. In general, extradural lesions are the most common with metastasis being the most frequent. Intradural tumors are rare, and the majority is extramedullary, with meningiomas and nerve sheath tumors being the most frequent. Intramedullary tumors are uncommon spinal tumors. Astrocytomas and ependymomas comprise the majority of the intramedullary tumors. The most important tumors are documented with appropriate high quality CT or MR images and the characteristics of these tumors are also summarized in a comprehensive table. Finally we illustrate the use of the new World Health Organization (WHO) classification of neoplasms affecting the central nervous system

  19. MRI of infections and neoplasms of the spine and spinal cord in 55 patients with AIDS

    International Nuclear Information System (INIS)

    Thurnher, M.M.; Post, M.J.D.; Jinkins, J.R.

    2000-01-01

    Our purpose was to describe the range of MRI findings in infectious and neoplastic involvement of the spine and spinal cord in symptomatic patients with the acquired immunodeficiency syndrome (AIDS). MRI studies in 55 patients with AIDS and neurological signs and symptoms thought to be related to the spine or spinal cord were reviewed. We categorized the findings according to the spinal compartment involved. There were 29 patients with extradural, 11 with intradural-extramedullary and 9 with intramedullary disease. In 6 patients more than one compartment was involved simultaneously, and patients presented with multiple lesions in the same compartment. The most common causes of extradural disease were bone lesions (28); an epidural mass was seen in 14 and spondylodiscitis in 4 patients. Cytomegalovirus polyradiculitis was the most common cause of intradural-extramedullary disease (in 10 cases); herpes radiculitis was seen in two, and tuberculous infection in another two. In three cases leptomeningeal contrast enhancement was due to lymphoma. Human immunodeficiency virus (HIV) myelitis was seen in two patients, presumed vacuolar myelopathy in two, toxoplasma myelitis in four, intramedullary lymphoma in one, and herpes myelitis in one. Familiarity with the various potential pathological entities that can affect the spine and spinal cord in the AIDS population and their imaging characteristics is crucial for initiation of further diagnostic tests and appropriate medical or surgical treatment. (orig.)

  20. Quantitative and qualitative temporal evolution of gadolinium enhancement of spinal lesion

    International Nuclear Information System (INIS)

    Sze, G.; Krol, G.

    1988-01-01

    Seventy gadolinium-enhanced studies of spinal lesions were reviewed, and 34 were selected for analysis of the temporal quantitative and qualitative aspects of enhancement. Thirteen patients had intradural extramedullary lesions, eight had intramedullary lesions, and 13 had extradural lesions. Data analysis was conducted by measuring intensities of normal and pathologic tissues on short repetition-time (TR) scans. The evolution of enhancement of spinal tumors was found to differ, depending on which compartment the tumor was located. Intradural extramedullary nodules showed their most prominent enhancement on immediate postcontrast scans. Extradural lesions varied in behavior. Some demonstrated an increase in enhancement, while others showed a decrease. Intramedullary tumors usually showed an increase in enhancement on delayed scans. In most cases, however, the quantitative increase was minor. The exception occurred in a case of necrotic cord glioma. Immediate postcontrast short-TR scans will nearly always be sufficient for the evaluation of suspected spinal lesions. Only the very occasional case may benefit from delayed scans

  1. Hemangiopericytoma invading the craniovertebral junction: First reported case and review of the literature

    Directory of Open Access Journals (Sweden)

    Doniel Drazin

    2013-01-01

    Full Text Available Occurrence of hemangiopericytoma (HPC in the central nervous system is rare. Spinal HPCs with intramedullary involvement are even more unusual. We present a case of a craniovertebral intradural HPC with both extra- and intra-medullary extensions. Though the patient presented with vague cervical symptoms, imaging was indicative of an intradural lesion from the occiput to C4 and a second smaller, subclinical lesion, at the T2-3 level. He underwent gross total surgical resection of the craniovertebral lesion and did well post-operatively. The thoracic lesion was treated with radiosurgery and the patient is neurologically at baseline 5 years later. Gross total resection of HPCs is the recommended treatment when possible. Histopathology is crucial for diagnosis due to both its rarity and similar characteristics to other tumors on physical and radiographic evaluations. Recognizing that these uncommon tumors can occur with both extra-medullary and intra-medullarly locations are important for diagnosis and treatment recommendations. Future studies using national surgical databases that contain histology will be needed to understand the long-term clinical outcomes.

  2. MRI of infections and neoplasms of the spine and spinal cord in 55 patients with AIDS

    Energy Technology Data Exchange (ETDEWEB)

    Thurnher, M.M. [Neuroradiology Section, Department of Radiology, University Hospital Vienna (Austria); Post, M.J.D. [Department of Radiology, Neuroradiology Section, University of Miami School of Medicine, Miami, FL (United States); Jinkins, J.R. [Neuroimaging Research, Department of Radiology, Nebraska Medical Center, Omaha, NE (United States)

    2000-08-01

    Our purpose was to describe the range of MRI findings in infectious and neoplastic involvement of the spine and spinal cord in symptomatic patients with the acquired immunodeficiency syndrome (AIDS). MRI studies in 55 patients with AIDS and neurological signs and symptoms thought to be related to the spine or spinal cord were reviewed. We categorized the findings according to the spinal compartment involved. There were 29 patients with extradural, 11 with intradural-extramedullary and 9 with intramedullary disease. In 6 patients more than one compartment was involved simultaneously, and patients presented with multiple lesions in the same compartment. The most common causes of extradural disease were bone lesions (28); an epidural mass was seen in 14 and spondylodiscitis in 4 patients. Cytomegalovirus polyradiculitis was the most common cause of intradural-extramedullary disease (in 10 cases); herpes radiculitis was seen in two, and tuberculous infection in another two. In three cases leptomeningeal contrast enhancement was due to lymphoma. Human immunodeficiency virus (HIV) myelitis was seen in two patients, presumed vacuolar myelopathy in two, toxoplasma myelitis in four, intramedullary lymphoma in one, and herpes myelitis in one. Familiarity with the various potential pathological entities that can affect the spine and spinal cord in the AIDS population and their imaging characteristics is crucial for initiation of further diagnostic tests and appropriate medical or surgical treatment. (orig.)

  3. Cavernous angioma of the cauda equina: case report Angioma cavernoso de cauda equina: relato de caso

    Directory of Open Access Journals (Sweden)

    Asdrubal Falavigna

    2004-06-01

    Full Text Available We present a rare case of cavernous angioma of the cauda equina and review the eleven cases available in the literature. A 44-year-old woman presented with low back pain and sciatica associated with bowel and bladder dysfunction and motor weakness of the lower extremity. The MRI revealed an enhancing, heterogeneous and hyperintense intradural lesion compressing the cauda equina roots at the L4 level. Laminectomy at L3-L4 and total removal of the tumor were performed without additional neurological deficit. Pathology revealed a cavernous angioma. The literature, clinical presentation, technical examinations, and treatment are reviewed.Relatamos um caso de angioma cavernoso de cauda equina em mulher de 44 anos de idade com sintomas de lombociatalgia associada a fraqueza de membros inferiores e disfunção esfincteriana vesical e anal. Exame de ressonância magnética evidenciou lesão expansiva intradural heterogênea e hiperintensa na cauda eqüina. Indicado tratamento cirúrgico com remoção completa através de laminectomia L3 e L4. O exame anatomopatológico foi compatível com angioma cavernoso. Os onze casos encontrados na literatura são revisados correlacionando a apresentação clínica, tratamento proposto e prognóstico.

  4. EVIDENCE trial: design of a phase 2, randomized, controlled, multicenter study comparing flow diversion and traditional endovascular strategy in unruptured saccular wide-necked intracranial aneurysms.

    Science.gov (United States)

    Turjman, Francis; Levrier, Olivier; Combaz, Xavier; Bonafé, Alain; Biondi, Alessandra; Desal, Hubert; Bracard, Serge; Mounayer, Charbel; Riva, Roberto; Chapuis, Francois; Huot, Laure; Armoiry, Xavier; Gory, Benjamin

    2015-01-01

    Endovascular treatment of large, wide-necked intracranial aneurysms with coils is associated with low rates of initial angiographic occlusion and high rates of recurrence. The Pipeline™ Embolization Device has shown high rates of complete occlusion in uncontrolled clinical series. The study is a prospective, controlled, randomized, multicenter, phase 2 open-label trial. Intention-to-treat population includes age ≥18, unruptured saccular aneurysm located in the intra-dural area, neck diameter ≥4 and ≤10 mm, sac diameter ≥7 mm and ≤20 mm, "dome/neck" ratio is ≥1, diameter of the parent artery ≥2 mm and ≤5 mm, and no prior treatment of the aneurysm. Site can only participate if five patients have been previously treated with the Pipeline device. The primary end point of the study is complete occlusion of the aneurysm on angiogram performed 12 months after the endovascular procedure. Complete aneurysm occlusion is defined as the absence of visible blood flow, grade 1 according to the Raymond scale for the standard procedure group and grade 4 according to the grading scale of Kamran for the flow diverter group. The trial is currently enrolling and results of the data are pending the completion of enrollment and follow-up. This paper details the trial design of the French EVIDENCE phase 2 trial, a blinded, controlled randomized trial of wide-neck intra-dural aneurysms amenable to either traditional endovascular strategy or flow diversion with Pipeline device.

  5. Intracranial extension of spinal subarachnoid hematoma causing severe cerebral vasospasm.

    Science.gov (United States)

    Nam, Kyoung Hyup; Lee, Jae Il; Choi, Byung Kwan; Han, In Ho

    2014-12-01

    Spinal subarachnoid hemorrhages (SAH) can extend into the intracranial subarachnoid space, but, severe cerebral vasospasm is rare complication of the extension of intracranial SAH from a spinal subarachnoid hematoma. A 67-year-old woman started anticoagulant therapy for unstable angina. The next day, she developed severe back pain and paraplegia. MRI showed intradural and extramedullar low signal intensity at the T2-3, consistent with intradural hematoma. High signal intensity was also noted in the spinal cord from C5 to T4. We removed subarachnoid hematoma compressing the spinal cord. The following day, the patient complained of severe headache. Brain CT revealed SAH around both parietal lobes. Three days later, her consciousness decreased and left hemiplegia also developed. Brain MRI demonstrated multiple cerebral infarctions, mainly in the right posterior cerebral artery territory, left parietal lobe and right watershed area. Conventional cerebral angiography confirmed diffuse severe vasospasm of the cerebral arteries. After intensive care for a month, the patient was transferred to the rehabilitation department. After 6 months, neurologic deterioration improved partially. We speculate that surgeons should anticipate possible delayed neurological complications due to cerebral vasospasm if intracranial SAH is detected after spinal subarachnoid hematoma.

  6. Endoscopic transphenoidal approach for fibrous dysplasia of clivus, tuberculum sellae and sphenoid sinus; report of three cases.

    Science.gov (United States)

    Anik, Ihsan; Koc, Kenan; Cabuk, Burak; Ceylan, Savas

    2012-01-01

    Fibrous dysplasia is a benign disease in which medullary bone is replaced by fibro-osseous tissue, and causes distortion and overgrowth of the involved bone and represents about 3% of all bone tumors. There is variability in the manifestation of Fibrous Dysplasia lesions with imaging techniques due to their proportional variations of fibrous to osseous tissue. Radiological differential diagnoses include meningioma, chordoma on MRI and Paget disease on CT imagings. We report three cases of monostotic fibrous dysplasia, affecting clivus, tuberculum sellae, and sphenoid sinus through the pterygopalatine fossa. We performed pure endoscopic transphenoidal approach for those three cases with the guide of neuronavigation. Extended approach was used via binostril for fibrous dysplasia of clivus and Tuberculum sellae. Radiologic report revealed chordoma and meningioma for the two cases and fibrous dysplasia for the last case. Total resection of tuberculum sellae, subtotal resection of clivus and partial resection of sphenoid sinus lesions were performed. Pathology diagnosis were fibrous dysplasia in all three patients. Histopathological sampling provided by surgical approach should be obtained to establish the final diagnosis. Endoscopic approaches are convenient for skull base lesions even for biopsy or curative resections providing panoramic view and avoid brain retraction.

  7. The presence and absence of lymphatic vessels in the adult human intervertebral disc: relation to disc pathology

    Energy Technology Data Exchange (ETDEWEB)

    Kliskey, Karolina; Williams, Kelly; Yu, J.; Urban, Jill; Athanasou, Nick [University of Oxford, Nuffield Department of Orthopaedic, Rheumatology and Musculoskeletal Science, Oxford (United Kingdom); Jackson, David [Weatherall Institute of Molecular Medicine, Human Immunology Unit, Oxford (United Kingdom)

    2009-12-15

    Although the normal adult human intervertebral disc is considered to be avascular, vascularised cellular fibrous tissue can be found in pathological conditions involving the disc such as disc herniation. Whether lymphatics vessels form a component of this reparative tissue is not known as the presence or absence of lymphatics in herniated and normal disc tissue is not known. We examined spinal tissues and discectomy specimens for the presence of lymphatics. The examination used immunohistochemistry to identify the specific lymphatic endothelial cell markers, podoplanin and LYVE1. Lymphatic vessels were not found in the nucleus pulposus or annulus fibrosus of intact, non-herniated lumbar and thoracic discs but were present in the surrounding ligaments. Ingrowth of fibrous tissue was seen in 73% of herniated disc specimens of which 36% contained LYVE1+/podoplanin + lymphatic vessels. Lymphatic vessels were not seen in the sacrum and coccyx or biopsies of four sacrococcygeal chordomas, but they were noted in surrounding extra-osseous fat and fibrous tissue at the edge of the infiltrating tumour. Our findings indicate that lymphatic vessels are not present in the normal adult intervertebral disc but that, when there is extrusion of disc material into surrounding soft tissue, there is ingrowth of reparative fibrous tissue containing lymphatic vessels. Our findings also indicate that chordoma, a tumour of notochordal origin, spreads to regional lymph nodes via lymphatics in para-spinal soft tissues. (orig.)

  8. Brain Metastasis in Bone and Soft Tissue Cancers: A Review of Incidence, Interventions, and Outcomes

    Directory of Open Access Journals (Sweden)

    Faris Shweikeh

    2014-01-01

    Full Text Available Bone and soft tissue malignancies account for a small portion of brain metastases. In this review, we characterize their incidence, treatments, and prognosis. Most of the data in the literature is based on case reports and small case series. Less than 5% of brain metastases are from bone and soft tissue sarcomas, occurring most commonly in Ewing’s sarcoma, malignant fibrous tumors, and osteosarcoma. Mean interval from initial cancer diagnosis to brain metastasis is in the range of 20–30 months, with most being detected before 24 months (osteosarcoma, Ewing sarcoma, chordoma, angiosarcoma, and rhabdomyosarcoma, some at 24–36 months (malignant fibrous tumors, malignant peripheral nerve sheath tumors, and alveolar soft part sarcoma, and a few after 36 months (chondrosarcoma and liposarcoma. Overall mean survival ranges between 7 and 16 months, with the majority surviving < 12 months (Ewing’s sarcoma, liposarcoma, malignant fibrous tumors, malignant peripheral nerve sheath tumors, angiosarcoma and chordomas. Management is heterogeneous involving surgery, radiosurgery, radiotherapy, and chemotherapy. While a survival advantage may exist for those given aggressive treatment involving surgical resection, such patients tended to have a favorable preoperative performance status and minimal systemic disease.

  9. [Endonasal skull base endoscopy].

    Science.gov (United States)

    Simal-Julián, Juan Antonio; Miranda-Lloret, Pablo; Pancucci, Giovanni; Evangelista-Zamora, Rocío; Pérez-Borredá, Pedro; Sanromán-Álvarez, Pablo; Perez-de-Sanromán, Laila; Botella-Asunción, Carlos

    2013-01-01

    The endoscopic endonasal techniques used in skull base surgery have evolved greatly in recent years. Our study objective was to perform a qualitative systematic review of the likewise systematic reviews in published English language literature, to examine the evidence and conclusions reached in these studies comparing transcranial and endoscopic approaches in skull base surgery. We searched the references on the MEDLINE and EMBASE electronic databases selecting the systematic reviews, meta-analyses and evidence based medicine reviews on skull based pathologies published from January 2000 until January 2013. We focused on endoscopic impact and on microsurgical and endoscopic technique comparisons. Full endoscopic endonasal approaches achieved gross total removal rates of craniopharyngiomas and chordomas higher than those for transcranial approaches. In anterior skull base meningiomas, complete resections were more frequently achieved after transcranial approaches, with a trend in favour of endoscopy with respect to visual prognosis. Endoscopic endonasal approaches minimised the postoperative complications after the treatment of cerebrospinal fluid (CSF) leaks, encephaloceles, meningoceles, craniopharyngiomas and chordomas, with the exception of postoperative CSF leaks. Randomized multicenter studies are necessary to resolve the controversy over endoscopic and microsurgical approaches in skull base surgery. Copyright © 2013 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

  10. A rare case of intraosseous benign notochordal cell tumor of the coccyx.

    Science.gov (United States)

    Uglialoro, Anthony D; Beebe, Kathleen S; Hameed, Meera; Benevenia, Joseph

    2009-06-01

    This article presents a case of a 53-year-old woman who presented with intermittent, dull, poorly localized lower back and buttock pain. The pain worsened in a seated position or after long periods of standing. A T1-weighted magnetic resonance image (MRI) of the sacrum and coccyx revealed a well-demarcated intraosseous lesion with homogeneous low signal intensity, while T2-weighted MRIs demonstrated homogeneous high signal intensity. An excisional biopsy revealed benign notochord cell tumor. The biopsy proved to be effective, as it relieved the patient's coccydynia. Due to the rarity of intraosseous benign notochordal cell tumors, it is essential to document and review this type of tumor. Only 2 benign notochordal cell tumors involving the coccyx have been previously reported, both of which presented with the same clinical symptoms of chronic coccydynia as our patient, likely due to the location of the involved lesion. The other leading diagnosis in our patient was chordoma, a malignant and locally aggressive neoplasm that is important to consider and exclude. Although chordomas have been well characterized in the surgery, pathology, and radiology literature, the benign notochordal cell tumor is a relative newcomer.

  11. The presence and absence of lymphatic vessels in the adult human intervertebral disc: relation to disc pathology

    International Nuclear Information System (INIS)

    Kliskey, Karolina; Williams, Kelly; Yu, J.; Urban, Jill; Athanasou, Nick; Jackson, David

    2009-01-01

    Although the normal adult human intervertebral disc is considered to be avascular, vascularised cellular fibrous tissue can be found in pathological conditions involving the disc such as disc herniation. Whether lymphatics vessels form a component of this reparative tissue is not known as the presence or absence of lymphatics in herniated and normal disc tissue is not known. We examined spinal tissues and discectomy specimens for the presence of lymphatics. The examination used immunohistochemistry to identify the specific lymphatic endothelial cell markers, podoplanin and LYVE1. Lymphatic vessels were not found in the nucleus pulposus or annulus fibrosus of intact, non-herniated lumbar and thoracic discs but were present in the surrounding ligaments. Ingrowth of fibrous tissue was seen in 73% of herniated disc specimens of which 36% contained LYVE1+/podoplanin + lymphatic vessels. Lymphatic vessels were not seen in the sacrum and coccyx or biopsies of four sacrococcygeal chordomas, but they were noted in surrounding extra-osseous fat and fibrous tissue at the edge of the infiltrating tumour. Our findings indicate that lymphatic vessels are not present in the normal adult intervertebral disc but that, when there is extrusion of disc material into surrounding soft tissue, there is ingrowth of reparative fibrous tissue containing lymphatic vessels. Our findings also indicate that chordoma, a tumour of notochordal origin, spreads to regional lymph nodes via lymphatics in para-spinal soft tissues. (orig.)

  12. C2 root nerve sheath tumors management.

    Science.gov (United States)

    El-Sissy, Mohamed H; Mahmoud, Mostafa

    2013-05-01

    Upper cervical nerve sheath tumors (NST) arising mainly from C2 root and to lesser extent from C1 root are not uncommon, they constitute approximately 5-12% of spinal nerve sheath tumors and 18-30% of all cervical nerve sheath tumors, unique in presentation and their relationship to neighbouring structures owing to the discrete anatomy at the upper cervical-craniovertebral region, and have atendency for growth reaching large-sized tumors before manifesting clinically due to the capacious spinal canal at this region; accordingly the surgical approaches to such tumors are modified. The aim of this paper is to discuss the surgical strategies for upper cervical nerve sheath tumors. Eleven patients (8 male and 3 females), age range 28-63 years, with C2 root nerve sheath tumors were operated upon based on their anatomical relations to the spinal cord. The magnetic resonance imaging findings were utilized to determine the surgical approach. The tumors had extra- and intradural components in 10 patients, while in one the tumor was purely intradural. The operative approaches included varied from extreme lateral transcondylar approach(n = 1) to laminectomy, whether complete(n = 3) a or hemilaminectomy(n = 7), with partial facetectomy(n = 7), and with suboccipital craniectomy(n = 2). The clinical picture ranged from spasticity (n = 8, 72,72 %), tingling and numbness below neck (n = 6, 54,54 %), weakness (n = 6, 54,54 %), posterior column involvement (n = 4, 26,36 %), and neck pain (n = 4, 36,36 %). The duration of symptoms ranged from 1 to 54 months, total excision was performed in 7 patients; while in 3 patients an extraspinal component, and in 1 patient a small intradural component, were left in situ. Eight patients showed improvement of myelopathy; 2 patients maintained their grades. One poor-grade patient was deteriorated. The surgical approaches for the C2 root nerve sheath tumors should be tailored according to the relationship to the spinal cord, determined by magnetic

  13. Histological effects of fibrin glue and synthetic tissue glues on the spinal cord: are they safe to use?

    Science.gov (United States)

    Kalsi, Pratipal; Thom, Maria; Choi, David

    2017-12-01

    Fibrin glues such as Tisseel ® have been established in neurosurgery for over thirty years. They are recommended for extradural use but have intradural applications. Brachial plexus reimplantation after trauma requires intradural fibrin glue because reimplanted nerves cannot be sutured to the spinal cord. Recently synthetic glues have become popular in spinal surgery but there is limited information about their safety. Our study compared the histological effects of Tisseel ® , Adherus ® and BioGlue ® on spinal cord using our rat brachial plexus repair model. Randomised observational animal study. Forty-one Sprague-Dawley rats divided in to control (n = 9), Tisseel ® (n = 8), BioGlue ® (n = 10) and Adherus ® (n = 14) groups. Under general anaesthesia a posterior midline cervical incision was made and hemi-laminectomies performed at C7 and T1. Dura was opened and T1 dorsal root transected and repositioned on the spinal cord. Two drops of Tisseel ® , BioGlue ® , Adherus ® or no glue (control) were applied over the cut nerve and cord. At days 7, 14 and 28 rats were euthanized, processed and sections stained with Haematoxylin & Eosin and evaluated blind by a neuropathologist. Control and Tisseel ® groups showed only mild focal inflammation in the cord. Adherus ® and Bioglue ® groups showed evidence of spinal cord inflammation and degeneration. All BioGlue ® and Adherus ® rats had evidence of distortion of the cord from the glue mass at all time points. Two BioGlue ® -treated and one Adherus ® -treated rat developed a hemiparesis. One BioGlue ® rat developed hind limb paralysis. One BioGlue ® rat failed to wake up at the end of the procedure. There were no complications in control and Tisseel ® groups. Tisseel ® caused a similar inflammatory response to control and may be used on spinal cord. BioGlue ® and Adherus ® should be applied thinly for a watertight dural closure but intradural use and contact with spinal tissue must be

  14. Contemporary analysis of the intraoperative and perioperative complications of neurosurgical procedures performed in the sitting position.

    Science.gov (United States)

    Himes, Benjamin T; Mallory, Grant W; Abcejo, Arnoley S; Pasternak, Jeffrey; Atkinson, John L D; Meyer, Fredric B; Marsh, W Richard; Link, Michael J; Clarke, Michelle J; Perkins, William; Van Gompel, Jamie J

    2017-07-01

    OBJECTIVE Historically, performing neurosurgery with the patient in the sitting position offered advantages such as improved visualization and gravity-assisted retraction. However, this position fell out of favor at many centers due to the perceived risk of venous air embolism (VAE) and other position-related complications. Some neurosurgical centers continue to perform sitting-position cases in select patients, often using modern monitoring techniques that may improve procedural safety. Therefore, this paper reports the risks associated with neurosurgical procedures performed in the sitting position in a modern series. METHODS The authors reviewed the anesthesia records for instances of clinically significant VAE and other complications for all neurosurgical procedures performed in the sitting position between January 1, 2000, and October 8, 2013. In addition, a prospectively maintained morbidity and mortality log of these procedures was reviewed for instances of subdural or intracerebral hemorrhage, tension pneumocephalus, and quadriplegia. Both overall and specific complication rates were calculated in relation to the specific type of procedure. RESULTS In a series of 1792 procedures, the overall complication rate related to the sitting position was 1.45%, which included clinically significant VAE, tension pneumocephalus, and subdural hemorrhage. The rate of any detected VAE was 4.7%, but the rate of VAE requiring clinical intervention was 1.06%. The risk of clinically significant VAE was highest in patients undergoing suboccipital craniotomy/craniectomy with a rate of 2.7% and an odds ratio (OR) of 2.8 relative to deep brain stimulator cases (95% confidence interval [CI] 1.2-70, p = 0.04). Sitting cervical spine cases had a comparatively lower complication rate of 0.7% and an OR of 0.28 as compared with all cranial procedures (95% CI 0.12-0.67, p < 0.01). Sitting cervical cases were further subdivided into extradural and intradural procedures. The rate of

  15. SU-E-T-626: Practical Method to Implement Arc Therapy Using Scanned Particle Beams

    Energy Technology Data Exchange (ETDEWEB)

    Eley, J; Mehta, M; Molitoris, J; Langner, U; Langen, K [University of Maryland School of Medicine, Baltimore, MD (United States)

    2015-06-15

    Purpose: The purpose of this study was to propose a method to implement arc therapy that is compatible with existing particle therapy systems having gantries and pencil-beam scanning capacities. Furthermore, we sought to demonstrate expected benefits of this method for selected clival chordoma patients. Methods: We propose that a desired particle arc treatment plan can be discretized into a finite number of fixed beams and that only one (or a subset) of these beams be delivered in any single treatment fraction; the target should receive uniform dose during each fraction. For 3 clival-chordoma patients, robust-optimized, scanned proton beams were simulated to deliver 78 Gy (RBE) to clinical target volumes (CTVs), using either a single-field plan with a posterior-anterior (PA) beam or a discrete-arc plan with 16 beams that were equally spaced throughout a 360-degree axial arc. Dose-volume metrics were compared with emphasis on the brainstem, since risk of radiation necrosis there can often restrict application of tumoricidal doses for chordomas. Results: The mean volume of brainstem receiving a dose of 60 Gy (RBE) or higher (V60Gy) was 10.3±0.9 cm{sup 3} for the single-field plan and 4.7±1.8 cm{sup 3} for the discrete-arc plan, a reduction of 55% in favor of arcs. The mean dose to the brainstem was also reduced using arcs, by 18%, while the maximum dose was nearly identical for both methods. For the whole brain, V60Gy was reduced by 23%, in favor of arcs. Mean dose to the CTVs were nearly identical for both strategies, within 0.3%. Conclusion: Discrete arc treatments can be implemented using existing scanned particle-beam facilities. Aside from the physical advantages, the biological uncertainties of particle therapy, particularly high in the distal edge, can be reduced by arc therapy via rotational smearing, which may be of benefit for tumors near the brainstem.

  16. Neurocutaneous melanocytosis presenting in a teenager: A case report and review of the literature.

    Science.gov (United States)

    Monica, I; Kumar, L Pavan; Uppin, Megha S; Jagannath Rao Naidu, Kotiyala V

    2015-01-01

    Neuro cutaneous melanocytosis (NCM) is a non-familial, congenital disorder characterized by multiple congenital nevi and brain or leptomeningeal abnormal melanin deposits. Here, we present an adult onset NCM. A 17-year-old boy presented with headache and double vision for 1 month. Magnetic resonance imaging of the brain showed hydrocephalus and abnormal meningeal hyper intensities in supra and infratentorial regions predominantly in the posterior fossa. Para medullary region showed an 11×10 mm nodular contrast enhancing nodule. Resection of an intramedullary central nervous system lesion revealed melanoma while skin biopsy was benign melanocytic nevus. As per Kadonaga and Frieden criteria, a diagnosis of NCM was made. Planned for craniospinal irradiation by three-dimensional conformal radiotherapy with a dose of 36 Gy, in 18 fractions (2 Gy/fraction and 5 days in a week) along with steroids however patient progressed and developed quadriplegia with intradural metastasis.

  17. Ancient schwannoma of thoracic spine in a schizophrenic patient with somatic delusion

    Directory of Open Access Journals (Sweden)

    Ya-Ting Wen

    2016-03-01

    Full Text Available Ancient schwannoma is a rare variant of schwannoma characterized by histopathologic degenerative changes, which are thought to be the result of long-term tumor growth and aging. However, ancient schwannoma in the spinal canal is particularly rare. We report a case of thoracic spine intradural extramedullary ancient schwannoma in a schizophrenic patient, who kept saying that “something in his back was giving him electric shock” for a long time. Unfortunately, this complaint was misinterpreted as somatic delusion symptoms. A spinal cord tumor was taken into consideration only after paraparesis developed. We have highlighted this case to remind every clinician to remain alert about the possibility of organic disease while treating patients with psychotic disorder history. Thorough neurological examination is required to avoid misdiagnosis. Spinal canal schwannoma can be totally removed successfully with good functional outcome and prognosis.

  18. [Limphomatous meningitis as recurrence site in Hodgkin's disease].

    Science.gov (United States)

    Greco, Martín; Valsecchi, Matías; Niccodemi, Cecilia; Presas, José; Corrado, Claudia; Winkel, Martín

    2006-01-01

    Intracraneal manifestations of Hodgkin's Disease (HD) are extremely rare, with an estimated incidence rate of approximately 0.5%. They can be classified as: 1) treatment-related leucoencephalopathy, 2) central nervous system infections, 3) paraneoplasic syndromes and 4) intracraneal lymphomas, which could be sub-classified into intraparenchymal or intradural masses. We describe a case of a 40 year-old male with mixed cellularity type HD who developed neurological manifestations as relapsed disease. Magnetic resonance imaging suggested leptomeningeal metastases and atypical cells were found in cerebrospinal fluid. The patient died from progressive disease refractory to third line chemotherapy. There are less than 50 similar cases reported in the literature. We review the clinical features and differential diagnosis of leptomeningeal metastases in Hodgkin's disease.

  19. Traumatic sacral pseudomeningocele with spina bifida occulta.

    Science.gov (United States)

    Banno, Tomohiro; Ohishi, Tsuyoshi; Suzuki, Daisuke; Honda, Yosuke; Kobayashi, Sho; Matsuyama, Yukihiro

    2012-01-01

    Pseudomeningocele arises after spinal fracture and nerve root avulsion or after complications of spine surgery. However, traumatic pseudomeningocele with spina bifida occulta is rare. In this report, a traumatic pseudomeningocele in a patient with spina bifida occulta that required surgical treatment is documented. This 37-year-old man presented to the authors' hospital with headache and a fluctuant mass in the center of his buttocks. A CT scan with myelography and MR imaging of the sacral region revealed a large subcutaneous area of fluid retention communicating with the intradural space through a defect of the S-2 lamina. Because 3 months of conservative treatment was unsuccessful, a free fat graft was placed with fibrin glue to seal the closure of the defect, followed by 1 week of CSF drainage. This is the first report on traumatic pseudomeningocele with spina bifida occulta successfully treated in this manner.

  20. Distinctive Surgical Strategy for Removal of String of Beadlike Schwannomas of Cauda Equina.

    Science.gov (United States)

    Liao, Dengyong; Zhang, Jing; Li, Dan; Deng, Xueyun; Ren, Qingqing; Chen, Haifeng

    2018-03-01

    Spinal schwannomas are the most common intradural extramedullary tumors. However, a string of beadlike schwannomas is rare. In some cases, the beadlike tumors might present a multiple segmental growing pattern, often located in the lumbar spinal canal and on 1 nerve fiber. Despite its benign nature, the resection of this string of beadlike tumors could be a challenge to neurosurgeons, especially when the tumors extend to a long distance. A 50-year-old female was admitted to our hospital, and her diagnosis was beadlike schwannomas. We performed 2 small hemilaminectomies and pulled all the tumors out gently. The patient made a full recovery quickly after resection. In this case, we first reported a new surgical approach for the removal of a string of beadlike cauda equina schwannomas. We hypothesize that this procedure would be a potential addition to the present surgical methods under some circumstances. Copyright © 2017 Elsevier Inc. All rights reserved.

  1. Pediatric intraspinal neoplasms

    International Nuclear Information System (INIS)

    Mueller, U.; Ahlhelm, F.; Ulmer, S.; Schlaeger, R.

    2014-01-01

    With an overall incidence of 10 % of all central nervous system tumors, spinal tumors are relatively rare in children. The majority of these tumors are astrocytomas and ependymomas (70 %) followed by rare non-glial tumor entities, such as gangliogliomas. They can be differentiated into intramedullary, extramedullary intradural and extramedullary extradural tumors according to their occurrence within the anatomical intraspinal compartments. The clinical presentation is generally unspecific. Longer lasting back pain or a gradually worsening scoliosis are often the first signs of the disease. Neurological deficits, such as gait disturbances and paresis often occur after a time delay. In rare cases increased intracranial pressure has been reported. Knowledge concerning potential organ manifestations, resulting complications and typical radiological presentation, especially in magnetic resonance imaging are mandatory for adequate diagnosis and treatment of affected patients. (orig.) [de

  2. Magnetic resonance imaging of intramedullary meningioma of the spinal cord: case report and review of the literature

    International Nuclear Information System (INIS)

    Covert, S.; Gandhi, D.; Goyal, M.; Woulfe, J.; Belanger, E.; Miller, W.; Modha, A.

    2003-01-01

    Intramedullary meningioma of the spinal cord is an extremely rare tumour. To the best of our knowledge, only 4 cases have been reported in the English literature, and limited information on imaging features is available. We present a further case and review the literature on this entity. Meningiomas are among the most common tumours that occur in the spinal canal, representing about 25% of all adult intraspinal tumours. They are commonly found in the thoracic segment of the spine and are most often intradural-extramedullary. Their location is explained by the way in which they develop. Meningiomas are derived from persistent arachnoid cell remnants in the spinal coverings, most commonly from arachnoid. They are usually adherent to, but do not arise from, dura. We present a case of intramedullary meningioma, a rare clinical entity, discuss the magnetic resonance imaging (MRI) findings and review the 4 cases that have been previously reported. (author)

  3. Meningioma of the scapula

    Energy Technology Data Exchange (ETDEWEB)

    Llauger, Jaume; Aixut, Sonia; Canete, Noemi; Palmer, Jaume; Sola, Marta [Universitat Autonoma de Barcelona, Department of Radiology, Hospital de la Santa Creu I Sant Pau, Barcelona (Spain); Bague, Silvia [Universitat Autonoma de Barcelona, Department of Pathology, Hospital de la Santa Creu I Sant Pau, Barcelona (Spain)

    2008-02-15

    Meningiomas account for approximately 15% of all intracranial tumors and are the most common non-glial primary tumors of the central nervous system. Most meningiomas are benign neoplasms with characteristic imaging features. Primary extradural meningiomas account for only 1-2% of all meningiomas. They must be differentiated from intradural meningiomas with secondary extradural extension and/or metastases. The vast majority of extradural meningiomas are found in the skull or in the head and neck region. We report on an extremely rare case of primary extradural meningioma that was located in the scapula. The lesion was resected. Radiographic findings and pathologic features are discussed. To the best of our knowledge, this form of presentation of an extradural meningioma has not been previously described. (orig.)

  4. MRI Findings of Juvenile Xanthogranuloma of the Spinal Cord: A Case Report

    International Nuclear Information System (INIS)

    Kim, Se Young; Park, Hee Jin; Lee, So Yeon; Chung, Eun Chul; Park, Hae Won; Kook, Shin Ho; Rho, Myung Ho; Goo, Ji Hye

    2013-01-01

    Juvenile xanthogranuloma (JXG) is a proliferative histiocytic disorder experienced during childhood and adolescents. JXG commonly presents as a solitary cutaneous lesion. Despite the term 'juvenile', development of the disease during adulthood is possible, although spinal JXG is extremely rare in adults. We describe a 67-year-old female patient who presented with an intradural-extramedullary (IDEM) tumor of the spinal cord. Magnetic resonance imaging (MRI) findings indicative of JXG of the spinal cord were seen, which was then confirmed pathologically. A lumbar spinal MRI with contrast enhancement showed an oval-shaped, well-defined IDEM tumor at the L1 level. This tumor had mixed signal intensity on the T1-weighted image and high signal intensity on the T2-weighted image. Central homogenous enhancement was observed after contrast administration.

  5. Sciatica as a presenting feature of thyroid follicular adenocarcinoma in a 79-year-old woman.

    LENUS (Irish Health Repository)

    Ogbodo, Elisha

    2011-01-01

    The authors describe an unusual case of metastatic thyroid follicular adenocarcinoma presenting with sciatica in a 79-year-old woman. The primary thyroid tumour was undiagnosed until this clinical presentation. The patient gave a short history of back pain and right-sided sciatica, which was progressive and nocturnal in nature. Neuroimaging revealed an enhancing intradural mass lesion, which was completely excised through a right L1-L3 hemilaminectomy. Histopathological examination of the excised tissue revealed a follicular thyroid carcinoma. Subsequent metastatic investigation revealed a heterogeneously attenuating mixed solid cystic mass in a retrosternal thyroid gland, with multiple solid pulmonary nodules suggestive of metastatic disease. She opted for palliative radiotherapy for the primary thyroid cancer and made remarkable postoperative improvement. The authors conclude that surgical treatment of solitary metastatic lesion may produce good symptomatic relief irrespective of patient\\'s age and primary pathology, while emphasising the need for detailed clinical evaluation of patients with \\'red flag\\' symptoms.

  6. Spinal CT scan, 2

    International Nuclear Information System (INIS)

    Nakagawa, Hiroshi

    1982-01-01

    Plain CT described fairly accurately the anatomy and lesions of the lumbar and sacral spines on their transverse sections. Since hernia of the intervertebral disc could be directly diagnosed by CT, indications of myelography could be restricted. Spinal-canal stenosis of the lumbar spine occurs because of various factors, and CT not only demonstrated the accurate size and morphology of bony canals, but also elucidated thickening of the joints and yellow ligament. CT was also useful for the diagnosis of tumors in the lumbar and sacral spines, visualizing the images of bone changes and soft tissues on the trasverse sections. But the diagnosis of intradural tumors required myelography and metrizamide CT. CT has become important for the diagnosis of spinal and spinal-cord diseases and for selection of the route of surgical arrival. (Chiba, N.)

  7. Spinal CT scan, 2. Lumbar and sacral spines

    Energy Technology Data Exchange (ETDEWEB)

    Nakagawa, Hiroshi (Aichi Medical Univ., Aichi (Japan))

    1982-08-01

    Plain CT described fairly accurately the anatomy and lesions of the lumbar and sacral spines on their transverse sections. Since hernia of the intervertebral disc could be directly diagnosed by CT, indications of myelography could be restricted. Spinal-canal stenosis of the lumbar spine occurs because of various factors, and CT not only demonstrated the accurate size and morphology of bony canals, but also elucidated thickening of the joints and yellow ligament. CT was also useful for the diagnosis of tumors in the lumbar and sacral spines, visualizing the images of bone changes and soft tissues on the trasverse sections. But the diagnosis of intradural tumors required myelography and metrizamide CT. CT has become important for the diagnosis of spinal and spinal-cord diseases and for selection of the route of surgical arrival.

  8. Meningitis tras anestesia espinal Meningitis after a spinal anesthesia

    OpenAIRE

    A. L. Vázquez-Martínez; F. Castro; G. Illodo; E. Freiré; M. A. Camba

    2008-01-01

    La meningitis post-punción es una importante complicación de la anestesia espinal. Describimos el caso de un varón de cuarenta y seis años que ingresó para tratamiento quirúrgico de una hernia umbilical, la cirugía se realizó bajo anestesia intradural. Tras la intervención el paciente comenzó con un cuadro clínico compatible con meningitis, que se confirmó tras examen del líquido cefalorraquídeo. Se trató con antibióticos a pesar de la no identificación de gérmenes, siendo la evolución favora...

  9. MR imaging of syringomyelia secondary to arachnoid adhesions

    International Nuclear Information System (INIS)

    Mark, A.S.; Andrews, B.; Sanches, J.; Manelfe, C.; Norman, D.; Newton, T.H.

    1987-01-01

    Arachnoid adhesions have been recognized as a cause of syringomyelia. Ten patients with surgically proven syringomyelia secondary to arachnoid adhesions (seven postraumatic, two postinfectious, one post-Pantopaque) were examined by MR imaging using sagittal and axial T1-weighted and sagittal T2-weighted sequences. A syrinx with loss of the sharp edge between the cord and the subarachnoid space as compared with the sharp interface seen in syringomyelia associated with Chiari I, was demonstrated on T1-weighted sequences in all patients. Intradural arachnoid cysts were found in nine patients. After surgical fenestration of the cyst, four patients improved and five remained unchanged. In one patient the syrinx decreased after fenestration of the cyst. Early detection and fesestration of these cavities may improve the patients' outcome

  10. Intracranial Vertebrobasilar Artery Dissection Associated with Postpartum Angiopathy

    Directory of Open Access Journals (Sweden)

    James S. McKinney

    2010-01-01

    Full Text Available Background. Cervicocephalic arterial dissection (CCAD is rare in the postpartum period. To our knowledge this is the first reported case of postpartum angiopathy (PPA presenting with ischemic stroke due to intracranial arterial dissection. Case. A 41-year-old woman presented with blurred vision, headache, and generalized seizures 5 days after delivering twins. She was treated with magnesium for eclampsia. MRI identified multiple posterior circulation infarcts. Angiography identified a complex dissection extending from both intradural vertebral arteries, through the basilar artery, and into both posterior cerebral arteries. Multiple segments of arterial dilatation and narrowing consistent with PPA were present. Xenon enhanced CT (Xe-CT showed reduced regional cerebral blood flow that is improved with elevation in blood pressure. Conclusion. Intracranial vertebrobasilar dissection causing stroke is a rare complication of pregnancy. Eclampsia and PPA may play a role in its pathogenesis. Blood pressure management may be tailored using quantitative blood flow studies, such as Xe-CT.

  11. Intracranial Vertebrobasilar Artery Dissection Associated with Postpartum Angiopathy

    Science.gov (United States)

    McKinney, James S.; Messé, Steven R.; Pukenas, Bryan A.; Satti, Sudhakar R.; Weigele, John B.; Hurst, Robert W.; Levine, Joshua M.; Kasner, Scott E.; Sansing, Lauren H.

    2010-01-01

    Background. Cervicocephalic arterial dissection (CCAD) is rare in the postpartum period. To our knowledge this is the first reported case of postpartum angiopathy (PPA) presenting with ischemic stroke due to intracranial arterial dissection. Case. A 41-year-old woman presented with blurred vision, headache, and generalized seizures 5 days after delivering twins. She was treated with magnesium for eclampsia. MRI identified multiple posterior circulation infarcts. Angiography identified a complex dissection extending from both intradural vertebral arteries, through the basilar artery, and into both posterior cerebral arteries. Multiple segments of arterial dilatation and narrowing consistent with PPA were present. Xenon enhanced CT (Xe-CT) showed reduced regional cerebral blood flow that is improved with elevation in blood pressure. Conclusion. Intracranial vertebrobasilar dissection causing stroke is a rare complication of pregnancy. Eclampsia and PPA may play a role in its pathogenesis. Blood pressure management may be tailored using quantitative blood flow studies, such as Xe-CT. PMID:20700423

  12. Upper cervical bronchogenic cyst: A rare condition at a rare location

    Directory of Open Access Journals (Sweden)

    Ranjeet Kumar Jha

    2018-01-01

    Full Text Available Intraspinal bronchogenic cyst (SBC is a rare but important cause of spinal cord compression, commonly seen in the cervicothoracic spine. We report a case of a 43-year-old male, presenting with complaints of neck pain, radiating to right shoulder, with numbness of right hand and fingers. Magnetic resonance imaging of the cervical spine revealed an intradural extramedullary, ventral cystic lesion extending from C2 to C4 vertebral levels. Complete surgical excision was performed, and the patient had a complete relief of symptoms postoperatively. Only 11 cases of SBCs have been reported in literature. We discuss the peculiar location of this lesion, possible embryological reasons and the overall surgical outcome of SBC.

  13. Holocord low grade astrocytoma - Role of radical irradiation and chemotherapy

    International Nuclear Information System (INIS)

    Goyal, S.; Puri, T.; Julka, R.K.

    2015-01-01

    Spinal intradural tumors, especially those extending along the entire length of the spinal cord, termed as ‘holocord’ tumors are uncommon. Most of these are gliomas, with astrocytomas (low grade) predominating in children and ependymomas in adults. Other histologies, though reported, are even rarer. Management is debatable, with both surgery and radiotherapy of such extensive tumors posing challenges. We describe a case of a 14-year-old girl with holocord astrocytoma extending from cervicomedullary junction till lumbar spine, who recovered full neurological function following radical irradiation of entire spine followed by temozolomide-based chemotherapy. No grade 3/4 bone marrow morbidity was seen. Five years following treatment, she maintained normal neurological function and apparently normal pubertal and skeletal growth despite residual disease visible on imaging. Literature review of existing reports of holocord astrocytomas highlighting management and outcome is presented.

  14. Primary multifocal gliosarcoma of the spinal cord

    Directory of Open Access Journals (Sweden)

    Ramesh M. Kumar

    2016-03-01

    Full Text Available Gliosarcoma (GS is a rare and exceedingly malignant neoplasm of the central nervous system. It displays clinical features similar to glioblastoma, yet is histologically unique as it harbors both gliomatous and sarcomatous cellular components. Involvement of the neuroaxis is predominantly limited to the cerebral parenchyma and meninges. Primary GS of the spinal cord is rarely encountered. We report a case of a 54 year old male who presented with 2 months of progressive, bilateral lower extremity sensory deficits. Magnetic resonance imaging of the neuro-axis revealed multiple intradural lesions involving the cervical and thoracic spinal cord without evidence of intracranial involvement. Surgical resection of a dural based, extramedullary cervical lesion and two exophytic, intramedullary thoracic lesions revealed gliosarcoma, WHO grade IV. The patient died approximately 11 months after presentation. This report confirms that GS is not limited to supratentorial involvement and can primarily affect the spinal cord.

  15. MR imaging findings in neurofibromatosis type 2

    International Nuclear Information System (INIS)

    Egelhoff, J.; Bates, D.; Ross, J.S.; Rothmer, A.D.

    1990-01-01

    This paper reports on neurofibromatosis 2(NF-2) (bilateral neurofibromatosis) that is an entity distinct from NF-1 (von Recklinghausen disease). While cranial MR findings have been described in these groups, there are no reports of the spinal findings in NF-2 separate from NF-1. Spinal MR findings in six NF-2 and six NF-1 patients were retrospectively reviewed. Sagittal T1-weighted images were obtained of the cervical (n = 10), thoracic (n = 9), and lumbar spine (n = 8) before and after administration of Gd-DTPA (0.1 mmol/kg). All NF-2 patients had enhancing intradural, extramedullary lesions (n = 5-35), consistent with neurofibromas or meningiomas. Three of six patients had enhancing intramedullary (IM) lesions, two of which were surgically confirmed as ependymomas

  16. Traumatic Acute Brain Herniation through the Ear in a Child; Concealed compound fracture

    Directory of Open Access Journals (Sweden)

    Rajeev Kariyattil

    2012-07-01

    Full Text Available A seven-year-old girl presented to Sultan Qaboos University Hospital, Oman, with a history of having been hit by a motor vehicle. After this, she had right-sided cerebrospinal fluid otorrhoea, and a week later, brain matter extruded through the right ear. A computed tomography scan of the brain demonstrated a tegmen fracture communicating with the external auditory canal. There was no hearing or facial nerve impairment and an otoscopic examination showed an intact tympanic membrane. She underwent a transcranial repair of the middle cranial fossa base, which revealed a wide dural and bony defect of the tegmen with herniation of the temporal lobe. Repair was made with an intradural patch of artificial dura. The rarity of this type of presentation of temporal bone fracture and its management are discussed.

  17. Thoracic spinal cord compression by a tophus.

    Science.gov (United States)

    Ntsiba, Honoré; Makosso, Edouard; Moyikoua, Armand

    2010-03-01

    We report a case of thoracic (T10) spinal cord compression by a tophus in a patient with known chronic gout. Spastic paraplegia developed gradually over 6 months in this 43-year-old man with hypertension, alcohol abuse, and chronic gouty arthritis with tophi. Magnetic resonance imaging and computed tomography visualized an intradural nodule measuring 1.5cm in diameter at the level of T10, as well as geodes in the left T10 lamina and left T9-T10 articular processes. The nodule was removed surgically and shown by histological examination to be a tophus. The neurological impairments resolved rapidly and completely. We found about 60 similar cases in the literature. Spinal cord compression in a patient with chronic gout can be caused by a tophus. Copyright 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  18. Cholesteatoma of the clivus.

    Science.gov (United States)

    Fassett, Daniel R; Kan, Peter; Chin, Steven S; Couldwell, William T

    2006-02-01

    Cholesteatomas (central nervous system epidermoids) can be found intradurally or extradurally in the central nervous system. Extradural intraosseous lesions are most commonly found in the petrous bone. The authors describe a unique case of a clival cholesteatoma in a 64-year-old woman who presented with headaches. No other neurological complaints or physical examination findings were noted. Magnetic resonance imaging showed an expansile lesion centered in the middle portion of the clivus. A large portion of the clivus was eroded. The lesion was explored via a transnasal trans-sphenoidal approach and granular debris was evacuated. The cystic lining was stripped from the surrounding bone, and the bone opening was widely fenestrated. Pathological examination showed keratinous debris with macrophages and an outer lining of benign epithelial tissue consistent with a cholesteatoma (epidermoid cyst). When surgically accessible, these lesions should be excised to prevent a recurrence. If inaccessible, marsupialization may be considered.

  19. Surgical treatment of lumbar extradural chondroma in a dog - case reportTratamento cirúrgico de condroma extradural lombar em cão - relato de caso

    Directory of Open Access Journals (Sweden)

    Paulo Vinícius Tertuliano Marinho

    2013-09-01

    Full Text Available Neoplasms originating in the spinal canal are uncommon in small animal veterinary clinic, but when present can cause significant neurological signs. Anatomically, these neoplasms can be classified as extradural, intradural-extramedullary and medullary. Extradural neoplasms are located outside the dura mater, but they can cause compression of the spinal cord. The chondromas are benign neoplasms characterized by the formation of cartilage and is rarely located in the spinal canal in both human and veterinary medicine. We describe a case of lumbar extradural chondroma in a dog that surgical decompression and removal of the mass allowed the resolution of clinical signs of paralysis with return of function of the hind limbs of the patient. As neoplasias com origem no canal medular são infrequentes na clínica veterinária de pequenos animais, porém quando presentes podem causar sinais neurológicos importantes. Anatomicamente estas neoplasias podem ser classificadas em extradural, intradural-extramedular e medular. As neoplasias extradurais estão localizadas fora da dura-máter, porém podem causar compressão da medula. Os condromas são neoplasias benignas, caracterizadas pela formação de tecido cartilaginoso sendo rara a sua localização no canal medular tanto em medicina humana como na medicina veterinária. O objetivo deste trabalho é descrever um caso de condroma extradural lombar em cão em que a descompressão cirúrgica e retirada da massa permitiu a resolução dos sinais clínicos de paralisia com retorno da função dos membros pélvicos do paciente.

  20. Clinical significance of nerve root enhancement in contrast-enhanced MR imaging of the postoperative lumbar spine

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Yeon Soo; Lee, Eun Ja; Kang, Si Won; Choi, Eun Seok [The Catholic Univ. of Korea, Taejon (Korea, Republic of); Song, Chang June; Kim, Jong Chul [Chungnam National Univ. School of Medicine, Taejon (Korea, Republic of)

    2001-09-01

    To determine the significance of nerve root contrast enhancement in patients with residual or recurrent symptomatic postoperative lumbar spine. Eighty-eight patients with 116 postoperative lumbar disc lesions causing radiating back pain underwent enhanced MR imaging. Intradural nerve root enhancement was quantified by pixel measurement, and affected nerve roots were compared before and after contrast administration. Extradural nerve root enhancement was assessed visually, and nerve root enhancement and clinical symptoms were correlated. Associated lesions such as recurrent disc herniation, scar tissue, nerve root thickening and nerve root displacement were also evaluated. Of 26 cases (22.4%) involving intradural nerve root enhancement, 22 (84.6%) showed significant clinical symptoms (p=0.002). and of 59 (50.9%) demonstrating extradural enhancement, clinical symptoms showed significant correlation in 47 (79.7%) (p=0.001). Nerve root enhancement, including eleven cases where this was both intra-and extradural, showed highly significant association with clinical symptoms in 74 of the 116 cases (63.8%) (p=0.000). Among 33 cases (28.4%) of recurrent disc herniation, nerve root enhancement was observed in 28 (84.8%) and in 24 of these 28 (85.7%), significant correlation with clinical symptoms was observed (p=0.000). Where epidural fibrosis was present, correlation between nerve root enhancement and clinical symptoms was not significant (p>0.05). Nerve root thickening and displaced nerve root were, however, significantly associated with symptoms (87.2% and 88.6%, respectively). In patients with postoperative lumbar spine, the association between nerve root enhancement revealed by MRI and clinical symptoms was highly significant.

  1. MRI EVALUATION OF SPINAL CORD TUMOURS WITH HISTOPATHOLOGICAL CORRELATION

    Directory of Open Access Journals (Sweden)

    Ashok Srikar Chowdhary

    2017-12-01

    Full Text Available BACKGROUND Spinal cord tumours are relatively rare tumours and can present with a wide variety of symptoms. If they are not diagnosed early and treated immediately, they can lead to neurological deficits and disability. Therefore, accurate diagnosis is necessary, which will help in directing the therapy. Nowadays, MRI is the most commonly used modality for spinal cord tumour diagnosis unless there is a contraindication. The aim of this study was to study the demographic profile of patients with spinal cord tumours to assess the distribution, features, localisation and extent of spinal cord tumours by MRI and correlate the tissue characterisation by MRI with that of histopathological examination. MATERIALS AND METHODS A prospective study was conducted in the Departments of Radiodiagnosis, Neurosurgery and Pathology at SCBMCH, Cuttack, from October 2010 to October 2012. 52 patients diagnosed as having spinal cord tumours by clinical examination and MRI were followed till post-surgery discharge. RESULTS Out of the 52 patients with spinal cord tumours, 28 patients (54% were males and females made up around 46% (24 patients. Around 6% of the patients were in the paediatric age group. Our study showed that intradural extramedullary tumours 36/52 (69% were the commonest followed by intramedullary tumours 10/52 (19% and extradural tumours 6/52 (12%. Overall, schwannoma was the commonest spinal cord tumour accounting for 46.1% of the tumours. Out of 52 cases, MRI diagnosed 46 cases (88.46% correctly and misdiagnosed 6 cases. MRI was able to correctly diagnose 91.67% of the intradural extramedullary tumours, 90% of the intramedullary tumours and 66.67% of the extradural tumours. CONCLUSION MRI is the preoperative investigation of choice in the evaluation of spinal cord tumours. MRI can accurately diagnose spinal tumours and guide surgical resection.

  2. MR myelography

    Energy Technology Data Exchange (ETDEWEB)

    Kwag, Hyon Joo; Choi, Hye Young; Han, Yoo Mie; Lim, Soo Mee; Lee, Sun Wha [College of Medicine, Ewha Womans University, Seoul (Korea, Republic of)

    1995-10-15

    We performed this study to describe the findings of MR Myelography (MRM) of herniated disc disease, spinal stenosis and spinal tumor and to evaluate this usefulness of the MRM in comparison to MRI. MRI and MRM were performed in 31 patients with herniated disc disease (12 patients), spinal stenosis (11 patients) and spinal tumor (8 patients). MRI and MRM were done with 1.5-T Signa MR, using fat suppressed heavily T2-weighted fast spin echo technique. We retrospectively analyzed MRM images about the thecal sac indentation, compression or displacement of the nerve root, extent and degree of narrowing of spinal canal, relationship between spinal tumor and spinal cord. MRM findings were compared with MRI in all cases. In 18 herniated disc cases of 12 patients, focal filling defect with cutoff or displacement of the nerve root in eight of paracentral herniated disc was seen. Cutoff and displacement of the nerve root were more clearly delineated on MRM than MRI. In the patients of spinal stenosis (11 cases), hourglass deformity of the thecal sac or complete spinal block of the subarachnoid space was clearly demonstrated. The extent and severity of spinal stenosis were more accurately evaluated on MRM than MRI. MRM finding of intramedullary tumor (3 cases) was enlargement of spinal cord. Five cases of intradural extramedullary tumor showed intradural filling defect, which caused contralateral displacement of the spinal cord with meniscus sign on inferior margin of the mass. MRM shows characteristic findings of herniated disc disease, spinal stenosis and spinal tumor. MRM yields excellent definition of the thecal sac, nerve roots and nerve root sleeves in relation to herniated disc and may be more accurate in evaluation of the degree and extent of spinal stenosis than MRI.

  3. Outcome of endovascular treatment in symptomatic intracranial vascular stenosis

    International Nuclear Information System (INIS)

    Suh, Dae Chul; Kim, Sang Joon; Lee, Duk Hee; Kim, Won; Choi, Choong Gon; Lee, Jeong Hyun; Kwon, Sun Uck; Kim, Jong Sung; Kim, Hyun Jeong

    2005-01-01

    The outcome evaluation for the revascularization of intracranial vascular stenoses has not been fully described due to the highly technical nature of the procedure. We report here on the early and late clinical outcome of angioplasty and/or stenting of symptomatic severe intracranial vascular stenoses at a single institute. Since 1995, we have treated 35 patients with symptomatic intracranial vascular stenosis (more than 70% stenosis, mean stenosis: 78.6% ± 6.2%). Angioplasty (n = 19) was performed for the horizontal segment of the middle cerebral artery (M1) (n = 16) and the basilar artery (BA) (n = 1), the intradural vertebral artery (VA) (n = 1), and the cavernous internal carotid artery (ICA) (n = 1). Stenting (n = 16) was performed for the cavernous or petrous ICAs (n = 9), the intradural VA (n = 3), BA (n = 2), and M1 (n = 2) artery. We assessed the angiographic success (defined as residual stenosis < 50%) rate, the periprocedural complications during the 30-day periprocedural period, the symptomatic recurrence and restenosis during a mean 22-month follow-up (FU) period. The Kaplan-Meier estimate of the cumulative even-free rate of the major cerebrovascular events, i.e. death, stroke or restenosis, was also done. Angiographic success was achieved in 97% of our patients (34/35). There were four procedure-related complications (11%) including a death and a minor stroke. During the mean 22-month FU, the asymptomatic restenosis rate was 9% and the symptomatic restenosis rate was 6% in the target lesion and 9% in all the vascular territories. The Kaplan-Meier estimate was 70.6% (95% confidence interval = 46.5-94.7) after 33 month of FU. In addition to a high angiographic success rate and an acceptable periprocedural complication rate, intracranial angioplasty and/or stenting revealed a relatively low symptomatic recurrence rate. Hemorrhage is a rare, but the physician must aware that potentially fatal periprocedural complications can occur

  4. P12.01 Epidemiology in spinal tumors treated surgically at the South Central Hospital of High Specialty from PEMEX in Mexico

    Science.gov (United States)

    Hernandez Resendiz, R.; Cordoba Mosqueda, M.; Guerra Mora, J.; Loya Aguilar, I.; Garcia Gonzalez, U.

    2016-01-01

    Abstract Introduction: The spinal tumors are rare neoplasms, they can be primary or metastatic; in the literature they are divided in extradural and intradural, extramedullary and intramedullary, from which extradural tumors are the most frequent and are usually metastatic, the intramedullary are generally gliomas. From the primary tumors up to 78% are benign and 22% malign, the histological stripe and the involvement to the spinal compartments are of great importance for the results and the treatment which is mainly surgical, individualized and meticulously planned with the support of technological resources such as the electrophysiological monitoring during the surgery. Methods and Materials: Observational study with a range of patients from March 1999- March 2016 to whom surgical resection of the spinal tumor was performed and reported on the Electronic Files of the South Central Hospital of High Specialty PEMEX. A Statistical analysis is made with the SPSS Statistic of disease of the Institution program. Results: 23 patients with spinal tumor surgical resection were found. The median age was 53 ± 10 years. The most common clinical manifestation was radiculopathy (65%). The Karnofsky scale was used for initial evaluation where a 43% of patients had a 90 score at the moment of the diagnosis, while 65% had an ECOG 1. The most frequent tumor was the Spinal Shwannoma (39%), followed in prevalence by the Condroid Cordoma (17%), where the intradural extramedullary location was the most prevalent (78%). The medium rate of survival after the surgical procedure was from 11 months. Conclusions: Our cases and the international statistics coincide. Radiculopathy as high prevalence initial manifestation conceals us to dismiss in the sixth decade of life any possibility for spinal tumor presentation. Most of spinal tumor patients do not have any clinical deterioration in their basal state, which indicates that performing a successful surgical procedure and the right

  5. Clinical significance of nerve root enhancement in contrast-enhanced MR imaging of the postoperative lumbar spine

    International Nuclear Information System (INIS)

    Lee, Yeon Soo; Lee, Eun Ja; Kang, Si Won; Choi, Eun Seok; Song, Chang June; Kim, Jong Chul

    2001-01-01

    To determine the significance of nerve root contrast enhancement in patients with residual or recurrent symptomatic postoperative lumbar spine. Eighty-eight patients with 116 postoperative lumbar disc lesions causing radiating back pain underwent enhanced MR imaging. Intradural nerve root enhancement was quantified by pixel measurement, and affected nerve roots were compared before and after contrast administration. Extradural nerve root enhancement was assessed visually, and nerve root enhancement and clinical symptoms were correlated. Associated lesions such as recurrent disc herniation, scar tissue, nerve root thickening and nerve root displacement were also evaluated. Of 26 cases (22.4%) involving intradural nerve root enhancement, 22 (84.6%) showed significant clinical symptoms (p=0.002). and of 59 (50.9%) demonstrating extradural enhancement, clinical symptoms showed significant correlation in 47 (79.7%) (p=0.001). Nerve root enhancement, including eleven cases where this was both intra-and extradural, showed highly significant association with clinical symptoms in 74 of the 116 cases (63.8%) (p=0.000). Among 33 cases (28.4%) of recurrent disc herniation, nerve root enhancement was observed in 28 (84.8%) and in 24 of these 28 (85.7%), significant correlation with clinical symptoms was observed (p=0.000). Where epidural fibrosis was present, correlation between nerve root enhancement and clinical symptoms was not significant (p>0.05). Nerve root thickening and displaced nerve root were, however, significantly associated with symptoms (87.2% and 88.6%, respectively). In patients with postoperative lumbar spine, the association between nerve root enhancement revealed by MRI and clinical symptoms was highly significant

  6. Treatment of intracranial atherosclerotic arterial stenoses with a balloon-expandable cobalt chromium stent (Coroflex Blue): procedural safety, efficacy, and midterm patency.

    Science.gov (United States)

    Vajda, Zsolt; Miloslavski, Elina; Güthe, Thomas; Schmid, Elisabeth; Schul, Christoph; Albes, Guido; Henkes, Hans

    2010-07-01

    We evaluated the coronary balloon-expandable cobalt chromium stent Coroflex Blue for the treatment of intracranial atherosclerotic arterial stenoses (IAAS). Between March 2007 and October 2007, a total of 25 patients (20 male, age median 67 years) with 30 IAAS underwent endovascular treatment using Coroflex Blue stents (B. Braun, Germany). Location and degree of target stenoses before and after treatment and at follow-up and adverse clinical sequelae of treatment were registered. Angiographic follow-up was scheduled for 6, 12, 26, and 52 weeks after the treatment. The 30 treated lesions were located as follows: nine in intracranial-extradural internal carotid artery (ICA), three in intradural ICA, five in middle cerebral artery, eight in intradural vertebral artery, and five in basilar artery. The technical success rate was 100%. The degree of stenoses prior to and after treatment was 61 +/- 2% and 26 +/- 3% (mean +/- SE), respectively. A residual stenosis of <50% was achieved in 29 (97%) procedures. Treatment was uneventful in 28 out of 30 procedures (93%); one patient suffered a transient and one patient a permanent neurological deficit. Angiographic follow-up was available in all of the patients (100%) after 15.2 months (median) and showed significant (i.e., more than 50%) degree of recurrent stenosis in 11 (37%) of the lesions. Retreatment was performed in 11 (37%) lesions. The Coroflex Blue stent is easily inserted and safely deployed into intracranial arteries. The incidence of recurrent stenoses remains a concern. Stringent angiographic and clinical follow-up and retreatment are therefore mandatory.

  7. Treatment of intracranial atherosclerotic arterial stenoses with a balloon-expandable cobalt chromium stent (Coroflex Blue): procedural safety, efficacy, and midterm patency

    Energy Technology Data Exchange (ETDEWEB)

    Vajda, Zsolt; Miloslavski, Elina; Albes, Guido [Katharinenhospital - Klinikum Stuttgart, Klinik fuer Neuroradiologie, Stuttgart (Germany); Guethe, Thomas [Katharinenhospital - Klinikum Stuttgart, Klinik fuer Neuroradiologie, Stuttgart (Germany); Katharinenhospital Klinikum, Klinik fuer Neurologie, Stuttgart (Germany); Schmid, Elisabeth [Buergerhospital Klinikum, Klinik fuer Neurologie, Stuttgart (Germany); Schul, Christoph [Katharinenhospital Klinikum, Klinik fuer Neurochirurgie, Stuttgart (Germany); Henkes, Hans [Katharinenhospital - Klinikum Stuttgart, Klinik fuer Neuroradiologie, Stuttgart (Germany); Medizinische Fakultaet der Universitaet, Duisburg-Essen (Germany)

    2010-07-15

    We evaluated the coronary balloon-expandable cobalt chromium stent Coroflex Blue for the treatment of intracranial atherosclerotic arterial stenoses (IAAS). Between March 2007 and October 2007, a total of 25 patients (20 male, age median 67 years) with 30 IAAS underwent endovascular treatment using Coroflex Blue stents (B. Braun, Germany). Location and degree of target stenoses before and after treatment and at follow-up and adverse clinical sequelae of treatment were registered. Angiographic follow-up was scheduled for 6, 12, 26, and 52 weeks after the treatment. The 30 treated lesions were located as follows: nine in intracranial-extradural internal carotid artery (ICA), three in intradural ICA, five in middle cerebral artery, eight in intradural vertebral artery, and five in basilar artery. The technical success rate was 100%. The degree of stenoses prior to and after treatment was 61 {+-} 2% and 26 {+-} 3% (mean {+-} SE), respectively. A residual stenosis of <50% was achieved in 29 (97%) procedures. Treatment was uneventful in 28 out of 30 procedures (93%); one patient suffered a transient and one patient a permanent neurological deficit. Angiographic follow-up was available in all of the patients (100%) after 15.2 months (median) and showed significant (i.e., more than 50%) degree of recurrent stenosis in 11 (37%) of the lesions. Retreatment was performed in 11 (37%) lesions. The Coroflex Blue stent is easily inserted and safely deployed into intracranial arteries. The incidence of recurrent stenoses remains a concern. Stringent angiographic and clinical follow-up and retreatment are therefore mandatory. (orig.)

  8. Augmented reality-assisted skull base surgery.

    Science.gov (United States)

    Cabrilo, I; Sarrafzadeh, A; Bijlenga, P; Landis, B N; Schaller, K

    2014-12-01

    Neuronavigation is widely considered as a valuable tool during skull base surgery. Advances in neuronavigation technology, with the integration of augmented reality, present advantages over traditional point-based neuronavigation. However, this development has not yet made its way into routine surgical practice, possibly due to a lack of acquaintance with these systems. In this report, we illustrate the usefulness and easy application of augmented reality-based neuronavigation through a case example of a patient with a clivus chordoma. We also demonstrate how augmented reality can help throughout all phases of a skull base procedure, from the verification of neuronavigation accuracy to intraoperative image-guidance. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  9. Markers aiding the diagnosis of chondroid tumors: an immunohistochemical study including osteonectin, bcl-2, cox-2, actin, calponin, D2-40 (podoplanin), mdm-2, CD117 (c-kit), and YKL-40

    DEFF Research Database (Denmark)

    Daugaard, Søren; Christensen, Lise H; Høgdall, Estrid

    2009-01-01

    (s) for the different subgroups. Archival material from three extraskeletal myxoid chondrosarcomas, five chordomas, five chondromyxoid fibromas, five chondroblastomas and 25 chondrosarcomas was stained with antibodies against osteonectin, bcl-2, cox-2, actin, calponin, D2-40 (podoplanin), mdm-2, CD117 (c-kit) and YKL......-40. All 25 chondrosarcomas showed a positive staining reaction for D2-40, none for actin and CD117, and a partial reactivity for bcl-2 (36%). Chondroblastomas (5/5) and chondromyxoid fibromas (2/5) were the only tumors with a positive reaction for actin, and all chondroblastomas (n=5...... chondrosarcomas. A convincing immunoreactivity for calponin and/or actin in chondromyxoid fibromas and chondroblastomas may also be helpful in differentiating these tumors from chondrosarcomas....

  10. CT and MRI of sphenoid tumors and pseudo tumors; Scanographie et IRM des tumeurs et pseudo-tumeurs du sphenoide

    Energy Technology Data Exchange (ETDEWEB)

    Beaujeux, R.; Dietemann, J.L.; Brun, F.; Bourjat, P. [Centre Hospitalier Universitaire, 67 - Strasbourg (France)

    1994-12-31

    The radiological features of the main tumors and pseudotumors of the sphenoid bone with CT and MRI are described in detail. The lesions are classified as tumors and pseudotumors of the sphenoid bone and sphenoid sinus and as neighboring tumors and pseudotumors with secondary sphenoid invasion. Metastases, chondromas, fibrous dysplasia, myelomas, plasmocytomas and chordomas are the most frequent sphenoid lesions. Tumors and pseudotumors of the sphenoid sinus mainly include mucoceles and cholesterin granulomas. Neighboring tumors are of intracranial origin (pituitary adenoma, meningioma, aneurysm) or originate in the nasopharynx (nasopharyngeal fibroma, malignant tumors) or the nasosinusal cavities (inverted papillomas, invasive aspergillosis, malignant tumors). The radiological features do no always allow diagnosing the tumoral type. However, the merits of CT and, even more, of MRI for an accurate assessment of extension is indisputable. (authors). 41 refs., 12 figs.

  11. CT and MRI of sphenoid tumors and pseudo tumors

    International Nuclear Information System (INIS)

    Beaujeux, R.; Dietemann, J.L.; Brun, F.; Bourjat, P.

    1994-01-01

    The radiological features of the main tumors and pseudotumors of the sphenoid bone with CT and MRI are described in detail. The lesions are classified as tumors and pseudotumors of the sphenoid bone and sphenoid sinus and as neighboring tumors and pseudotumors with secondary sphenoid invasion. Metastases, chondromas, fibrous dysplasia, myelomas, plasmocytomas and chordomas are the most frequent sphenoid lesions. Tumors and pseudotumors of the sphenoid sinus mainly include mucoceles and cholesterin granulomas. Neighboring tumors are of intracranial origin (pituitary adenoma, meningioma, aneurysm) or originate in the nasopharynx (nasopharyngeal fibroma, malignant tumors) or the nasosinusal cavities (inverted papillomas, invasive aspergillosis, malignant tumors). The radiological features do no always allow diagnosing the tumoral type. However, the merits of CT and, even more, of MRI for an accurate assessment of extension is indisputable. (authors). 41 refs., 12 figs

  12. Parachordoma of Soft Tissues of the Arm: A Very Rare Tumour

    Directory of Open Access Journals (Sweden)

    Vicente Estrems Díaz

    2013-01-01

    Full Text Available Parachordoma is an infrequent neoplasm that bears some histologic resemblance to chordoma. It affects both sexes, occurs typically during the fourth decade of life, and tends to present as a slow-growing painless mass at the level of the soft tissues of the extremities. Diagnosis should be based on immunohistochemical and cytogenetic studies, as the findings of imaging techniques are often unspecific. Although it is considered a benign lesion, its behavior tends to be locally aggressive, with reports of a recurrence rate of up to 20% and of several cases of metastasis. Fewer than 60 cases have been published in the English-speaking literature. In this paper we present the case of a 32-year-old male with a two-year history of parachordoma in the right wrist.

  13. Isolated sphenoid sinus lesions: Experience with a few rare pathologies

    Directory of Open Access Journals (Sweden)

    Nishanth Sadashiva

    2017-01-01

    Full Text Available Introduction: The sphenoid sinus is often neglected because of its difficult access. The deep position of the sphenoid sinus hinders early diagnosis of pathologies in that location. Delayed diagnosis can cause serious complications due to proximity to many important structures. Objectives: The aim of this study is to demonstrate different pathologies which can affect the sphenoid sinus and elucidate the findings. Methods: Cases of isolated sphenoid sinus lesions encountered in the neurosurgical setting which had rare pathologies are discussed. Pathologies such as Langerhans cell histiocytosis, solitary plasmacytoma, chordoma, pituitary adenoma, leiomyosarcoma, fungal infection, and mucocele which appeared primarily in sphenoid sinus are discussed along with their imaging features and pathological findings. Conclusion: Multitude of different pathologies can occur in sphenoid sinus. Detailed preoperative imaging is very helpful, but transnasal biopsy and histological study are required often for definitive diagnosis. The possible advantages of early diagnosis before spread of pathology for prognosis cannot be overemphasized.

  14. Case report 351: Aggressive osteoblastoma of the third lumbar vertebra

    International Nuclear Information System (INIS)

    Abdelwahab, I.F.; Frankel, V.H.; Klein, M.J.

    1986-01-01

    In summary, a case is presented of an aggressive osteoblastoma affecting the third lumbar vertebra in a 65-year-old man. Because of the age of the patient, the aggressive, destructive nature of the tumor and the extensive involvement of the vertebral body, metastatic carcinoma as well as several primary malignancies were entertained in the differential diagnosis (e.g. plasmacytoma, chordoma, primary lymphoma of bone). However, the destructive, expanding nature of the lesion, the focal calcification within the area of destroyed bone, the production of new bone with dense sclerosis, raised the possibility of an osteoblastoma, despite the advanced age of the patient. Without the histological material, the type of osteoblastoma (aggressive) could not be diagnosed. (orig./SHA)

  15. Case report 351: Aggressive osteoblastoma of the third lumbar vertebra

    Energy Technology Data Exchange (ETDEWEB)

    Abdelwahab, I.F.; Frankel, V.H.; Klein, M.J.

    1986-02-01

    In summary, a case is presented of an aggressive osteoblastoma affecting the third lumbar vertebra in a 65-year-old man. Because of the age of the patient, the aggressive, destructive nature of the tumor and the extensive involvement of the vertebral body, metastatic carcinoma as well as several primary malignancies were entertained in the differential diagnosis (e.g. plasmacytoma, chordoma, primary lymphoma of bone). However, the destructive, expanding nature of the lesion, the focal calcification within the area of destroyed bone, the production of new bone with dense sclerosis, raised the possibility of an osteoblastoma, despite the advanced age of the patient. Without the histological material, the type of osteoblastoma (aggressive) could not be diagnosed. (orig./SHA).

  16. Arrested pneumatization of the sphenoid sinus mimicking intraosseous lesions of the skull base

    Energy Technology Data Exchange (ETDEWEB)

    Jalali, Elnaz; Tadinada, Aditya [Dept. of Oral and Maxillofacial Radiology, University of Connecticut School of Dental Medicine, Farmington (United States)

    2015-03-15

    Arrested pneumatization of the sphenoid sinus is a developmental variant that is not always well recognized and is often confused with other pathologies associated with the skull base. This report describes the case of a patient referred for cone-beam computed tomography (CBCT) imaging for dental implant therapy. CBCT demonstrated a well-defined incidental lesion in the left sphenoid sinus with soft tissue-like density and sclerotic borders with internal curvilinear opacifications. The differential diagnoses included intraosseous lipoma, arrested pneumatization of the sphenoid sinus, chondrosarcoma, chondroid chordoma, and ossifying fibroma. The radiographic diagnosis of arrested pneumatization was based on the location of the lesion, its well-defined nature, the presence of internal opacifications, and lack of expansion. Gray-scale CBCT imaging of the area demonstrated values similar to fatty tissue. This case highlighted the fact that benign developmental variants associated with the skull base share similar radiographic features with more serious pathological entities.

  17. MR imaging of the pelvis: a guide to incidental musculoskeletal findings for abdominal radiologists.

    Science.gov (United States)

    Gaetke-Udager, Kara; Girish, Gandikota; Kaza, Ravi K; Jacobson, Jon; Fessell, David; Morag, Yoav; Jamadar, David

    2014-08-01

    Occasionally patients who undergo magnetic resonance imaging for presumed pelvic disease demonstrate unexpected musculoskeletal imaging findings in the imaged field. Such incidental findings can be challenging to the abdominal radiologist, who may not be familiar with their appearance or know the appropriate diagnostic considerations. Findings can include both normal and abnormal bone marrow, osseous abnormalities such as Paget's disease, avascular necrosis, osteomyelitis, stress and insufficiency fractures, and athletic pubalgia, benign neoplasms such as enchondroma and bone island, malignant processes such as metastasis and chondrosarcoma, soft tissue processes such as abscess, nerve-related tumors, and chordoma, joint- and bursal-related processes such as sacroiliitis, iliopsoas bursitis, greater trochanteric pain syndrome, and labral tears, and iatrogenic processes such as bone graft or bone biopsy. Though not all-encompassing, this essay will help abdominal radiologists to identify and describe this variety of pelvic musculoskeletal conditions, understand key radiologic findings, and synthesize a differential diagnosis when appropriate.

  18. Benign notochordal cell tumors.

    Science.gov (United States)

    Martínez Gamarra, C; Bernabéu Taboada, D; Pozo Kreilinger, J J; Tapia Viñé, M

    Benign notochordal cell tumors (TBCN) are lesions with notochordal differentiation which affect the axial skeleton. They are characterized by asymptomatic or non-specific symptomatology and are radiologically unnoticed because of their small size, or because they are mistaken with other benign bone lesions, such as vertebral hemangiomas. When they are large, or symptomatic, can be differential diagnosis with metastases, primary bone tumors and chordomas. We present a case of a TBCN in a 50-year-old woman, with a sacral lesion seen in MRI. A CT-guided biopsy was scheduled to analyze the lesion, finding that the tumor was not clearly recognizable on CT, so the anatomical references of MRI were used to select the appropriate plane. The planning of the approach and the radio-pathological correlation were determinant to reach the definitive diagnosis. Copyright © 2017 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

  19. Vascular endothelial growth factor receptor 2 as a marker for malignant vascular tumors and mesothelioma: an immunohistochemical study of 262 vascular endothelial and 1640 nonvascular tumors.

    Science.gov (United States)

    Miettinen, Markku; Rikala, Maarit-Sarlomo; Rys, Janusz; Lasota, Jerzy; Wang, Zeng-Feng

    2012-04-01

    Vascular endothelial growth factor receptor 2 (VEGFR2) is a primary responder to vascular endothelial growth factor signal and thereby regulates endothelial migration and proliferation. This receptor is expressed in endothelial cells and in some vascular tumors, but many reports also detail its expression in carcinomas and lymphomas. VEGFR2 is a potential cell-type marker, and data on VEGFR2 expression may also have therapeutic significance in view of recent availability of VEGFR2 inhibitors. In this study, we immunohistochemically examined 262 vascular endothelial and 1640 nonvascular tumors and selected non-neoplastic tissues with a VEGFR2-specific rabbit monoclonal antibody 55B11. In early human embryo, VEFGR2 was expressed in endothelia of developing capillaries and in the thoracic duct, great vessels, hepatic sinusoids, epidermis, and mesothelia. In late first trimester fetus peripheral soft tissues, VEGFR2 was restricted to capillary endothelia, chondrocytes, and superficial portion of the epidermis. In normal adult tissues, it was restricted to endothelia and mesothelia. VEGFR2 was consistently expressed in angiosarcomas, Kaposi sarcomas, and retiform hemangioendotheliomas. It was detected in only half of epithelioid hemangioendotheliomas (15/27), usually focally. VEGFR2 was strongly expressed in most capillary hemangiomas and weakly or focally in cavernous, venous, and spindle cell hemangiomas and in lymphangiomas. Malignant epithelial mesothelioma was found to be a unique epithelial neoplasm with a strong and nearly consistent VEGFR2 expression, including membrane staining (35/38). Approximately 10% of squamous cell carcinomas and 23% of pulmonary adenocarcinomas contained focal positivity. The only nonendothelial mesenchymal tumors found to be VEGFR2 positive were biphasic synovial sarcoma (focal epithelial expression) and chordoma. All melanomas and lymphomas were negative. VEGFR2 is a promising marker for malignant vascular tumors and malignant

  20. Sparsity constrained split feasibility for dose-volume constraints in inverse planning of intensity-modulated photon or proton therapy

    Science.gov (United States)

    Penfold, Scott; Zalas, Rafał; Casiraghi, Margherita; Brooke, Mark; Censor, Yair; Schulte, Reinhard

    2017-05-01

    A split feasibility formulation for the inverse problem of intensity-modulated radiation therapy treatment planning with dose-volume constraints included in the planning algorithm is presented. It involves a new type of sparsity constraint that enables the inclusion of a percentage-violation constraint in the model problem and its handling by continuous (as opposed to integer) methods. We propose an iterative algorithmic framework for solving such a problem by applying the feasibility-seeking CQ-algorithm of Byrne combined with the automatic relaxation method that uses cyclic projections. Detailed implementation instructions are furnished. Functionality of the algorithm was demonstrated through the creation of an intensity-modulated proton therapy plan for a simple 2D C-shaped geometry and also for a realistic base-of-skull chordoma treatment site. Monte Carlo simulations of proton pencil beams of varying energy were conducted to obtain dose distributions for the 2D test case. A research release of the Pinnacle 3 proton treatment planning system was used to extract pencil beam doses for a clinical base-of-skull chordoma case. In both cases the beamlet doses were calculated to satisfy dose-volume constraints according to our new algorithm. Examination of the dose-volume histograms following inverse planning with our algorithm demonstrated that it performed as intended. The application of our proposed algorithm to dose-volume constraint inverse planning was successfully demonstrated. Comparison with optimized dose distributions from the research release of the Pinnacle 3 treatment planning system showed the algorithm could achieve equivalent or superior results.

  1. Initial clinical experience with scanned proton beams at the Italian National Center for Hadrontherapy (CNAO)

    Science.gov (United States)

    Tuan, J.; Vischioni, B.; Fossati, P.; Srivastava, A.; Vitolo, V.; Iannalfi, A.; Fiore, M.R.; Krengli, M.; Mizoe, J.E.; Orecchia, R.

    2013-01-01

    We report the initial toxicity data with scanned proton beams at the Italian National Center for Hadrontherapy (CNAO). In September 2011, CNAO commenced patient treatment with scanned proton beams within two prospective Phase II protocols approved by the Italian Health Ministry. Patients with chondrosarcoma or chordoma of the skull base or spine were eligible. By October 2012, 21 patients had completed treatment. Immobilization was performed using rigid non-perforated thermoplastic-masks and customized headrests or body-pillows as indicated. Non-contrast CT scans with immobilization devices in place and MRI scans in supine position were performed for treatment-planning. For chordoma, the prescribed doses were 74 cobalt grey equivalent (CGE) and 54 CGE to planning target volume 1 (PTV1) and PTV2, respectively. For chondrosarcoma, the prescribed doses were 70 CGE and 54 CGE to PTV1 and PTV2, respectively. Treatment was delivered five days a week in 35–37 fractions. Prior to treatment, the patients' positions were verified using an optical tracking system and orthogonal X-ray images. Proton beams were delivered using fixed-horizontal portals on a robotic couch. Weekly MRI incorporating diffusion-weighted-imaging was performed during the course of proton therapy. Patients were reviewed once weekly and acute toxicities were graded with the Common Terminology Criteria for Adverse Events (CTCAE). Median age of patients = 50 years (range, 21–74). All 21 patients completed the proton therapy without major toxicities and without treatment interruption. Median dose delivered was 74 CGE (range, 70–74). The maximum toxicity recorded was CTCAE Grade 2 in four patients. Our preliminary data demonstrates the clinical feasibility of scanned proton beams in Italy. PMID:23824124

  2. Radiation tolerance of the cervical spinal cord: incidence and dose-volume relationship of symptomatic and asymptomatic late effects following high dose irradiation of paraspinal tumors

    International Nuclear Information System (INIS)

    Liu, Mitchell C.C.; Munzenrider, John E.; Finkelstein, Dianne; Liebsch, Norbert; Adams, Judy; Hug, Eugen B.

    1997-01-01

    Purpose: Low grade chordomas and chondrosarcomas require high radiation doses for effective, lasting tumor control. Fractionated, 3-D planned, conformal proton radiation therapy has been used for lesions along the base of skull and spine to deliver high target doses, while respecting constraints of critical, normal tissues. In this study, we sought to determine the incidence of myelopathy after high dose radiotherapy to the cervical spine and investigated the influence of various treatment parameters, including dose-volume relationship. Methods and Materials: Between December 1980 and March 1996, 78 patients were treated at the Massachusetts General Hospital and Harvard Cyclotron Laboratory for primary or recurrent chordomas and chondrosarcomas of the cervical spine using combined proton and photon radiation therapy. In general, the tumor dose given was between 64.5 to 79.2 CGE (Cobalt Gray Equivalent). The guidelines for maximum permissible doses to spinal cord were: ≤ 64 CGE to the spinal cord surface and ≤ 53 CGE to the spinal cord center. Dose volume histograms of the spinal cord were analyzed to investigate a possible dose and volume relationship. Results: With a mean follow-up period of 46.6 months (range: 3 - 157 months), 4 of 78 patients (5.1%) developed high-grade (RTOG Grade 3 and 4) late toxicity: 3 patients (3.8%) experienced sensory deficits without motor deficits, none had any limitations of daily activities. One patient (1.2%) developed motor deficit with loss of motor function of one upper extremity. The only patient, who developed permanent motor damage had received additional prior radiation treatment and therefore received a cumulative spinal cord dose higher than the treatment guidelines. No patient treated within the guidelines experienced any motor impairment. Six patients (7.7%) experienced transient Lhermitt's syndrome and 1 patient (1.2%) developed asymptomatic radiographic MR findings only. Time to onset of symptoms of radiographic

  3. MULTIPLE SPINAL CANAL MENINGIOMAS

    Directory of Open Access Journals (Sweden)

    Nandigama Pratap Kumar

    2016-10-01

    Full Text Available BACKGROUND Meningiomas of the spinal canal are common tumours with the incidence of 25 percent of all spinal cord tumours. But multiple spinal canal meningiomas are rare in compare to solitary lesions and account for 2 to 3.5% of all spinal meningiomas. Most of the reported cases are both intra cranial and spinal. Exclusive involvement of the spinal canal by multiple meningiomas are very rare. We could find only sixteen cases in the literature to the best of our knowledge. Exclusive multiple spinal canal meningiomas occurring in the first two decades of life are seldom reported in the literature. We are presenting a case of multiple spinal canal meningiomas in a young patient of 17 years, who was earlier operated for single lesion. We analysed the literature, with illustration of our case. MATERIALS AND METHODS In September 2016, we performed a literature search for multiple spinal canal meningiomas involving exclusively the spinal canal with no limitation for language and publication date. The search was conducted through http://pubmed.com, a wellknown worldwide internet medical address. To the best of our knowledge, we could find only sixteen cases of multiple meningiomas exclusively confined to the spinal canal. Exclusive multiple spinal canal meningiomas occurring in the first two decades of life are seldom reported in the literature. We are presenting a case of multiple spinal canal meningiomas in a young patient of 17 years, who was earlier operated for solitary intradural extra medullary spinal canal meningioma at D4-D6 level, again presented with spastic quadriparesis of two years duration and MRI whole spine demonstrated multiple intradural extra medullary lesions, which were excised completely and the histopathological diagnosis was transitional meningioma. RESULTS Patient recovered from his weakness and sensory symptoms gradually and bladder and bowel symptoms improved gradually over a period of two to three weeks. CONCLUSION Multiple

  4. Human radicular veins: regulation of venous reflux in the absence of valves.

    Science.gov (United States)

    van der Kuip, M; Hoogland, P V; Groen, R J

    1999-02-01

    In the literature it is generally assumed that venous reflux within the radicular veins is prevented by the presence of bicuspid valves and narrowing of the transdural part of these vessels. Recently, we performed a human cadaver study of the internal vertebral venous plexus. Surprisingly, a large number of radicular and perimedullary veins appeared to be filled with Araldite CY 221 mixture, after injection of this material into the vertebral venous system, implicating reflux via the radicular veins and suggesting insufficiency of the presumed anti-reflux mechanism. Therefore, it was decided to study the radicular veins in order to determine and to investigate the presence or absence of anti-reflux mechanisms within this system. The vertebral venous systems of ten fresh human cadavers, between 64 and 93 years of age, were injected with Araldite CY 221 mixture. After polymerization, all cadavers were dissected and the spinal nerve sheaths, including nerve roots, radicular veins and epidural veins, were excised as a whole. After macroscopical examination, serial sections (40 microm) were cut on a freezing microtome and stained in Von Gieson medium. Every third section was stained immunohistochemically with smooth muscle antigen (SMA), to visualize smooth muscle cells. In all cadavers, a number of intradural radicular veins was filled with Araldite. Employing microscopical examination, no bicuspid valves were found. However, four structures were encountered that might serve as ananti-reflux-mechanism: 1) intravenous dural folds, 2) meandrous configuration, and 3) narrowing of the radicular veins at the point of penetration of the dura mater, and 4) varying numbers of smooth muscle fibers in the walls of the intradural and extradural parts of the radicular veins. Reflux via the radicular veins seems to be a physiological phenomenon. Structural valves have not been encountered during this study. Intravenous dural folds, meandrous configuration and narrowing of the

  5. Utility of nonpenetrating titanium clips for dural closure during spinal surgery to prevent postoperative cerebrospinal fluid leakage.

    Science.gov (United States)

    Ito, Kiyoshi; Aoyama, Tatsuro; Horiuchi, Tetsuyoshi; Hongo, Kazuhiro

    2015-12-01

    The nonpenetrating titanium clip has been successfully used in peripheral arterial bypass surgery. The purpose of this study was to evaluate the leakage pressures and patterns of nonpenetrating titanium clips using a simple model that mimicked spinal surgery. In addition, the authors describe their surgical experience with these clips and the follow-up results in 31 consecutive patients. The authors compared nonpenetrating titanium clips and expanded polytetrafluoroethylene (ePTFE) sutures in relation to the water pressure that could be tolerated by sutured ePTFE sheets, and the leakage pressure patterns were determined. The changes in leakage pressures at 5 minutes, 30 minutes, and 12 hours were examined when the clips and sutures were used in combination with the mesh-and-glue technique in an in vitro study. Thirty-one patients underwent spinal intradural procedures using nonpenetrating titanium clips to suture the dura maters using the meshand-glue technique, involving fibrin glue and polyglycolic acid-fibrin sheets. A significant difference was apparent between the ePTFE suture group and the nonpenetrating titanium clip group, with the latter showing a leakage pressure that could be sustained and was 1508% higher than that of the former (p = 0.001). In relation to leakage patterns, the nonpenetrating titanium clips did not make any suture holes in the ePTFE sheet and fluid leakage occurred between the clips, whereas fluid leakage was associated with the pressure elevation that occurred at the suture holes made by the ePTFE sutures. Of the 31 patients who underwent spinal intradural procedures using nonpenetrating titanium clips, 1 (3.2%) experienced cerebrospinal fluid (CSF) leakage postoperatively. No other complications-for example, allergic reactions, adhesions, or infections--were encountered. The interrupted placement of nonpenetrating titanium clips enables dural closure without creating any holes. These clips facilitate improvements in the initial

  6. Paraganglioma na coluna vertebral: relato de caso Paraganglioma in the spine: case report

    Directory of Open Access Journals (Sweden)

    Maurício Santos Gusmão

    2012-04-01

    Full Text Available Os paragangliomas são tumores neuroendócrinos raros com origem em células especializadas derivadas da crista neural, acometendo a região da carótida ou jugulotimpânica. São raros dentro do canal vertebral, e quando ocorrem são normalmente encontrados no compartimento intradural e extramedular da região lombossacra. Este relato apresenta o caso de um paciente de 26 anos, sexo masculino, com diagnóstico de fratura patológica da coluna torácica (T10, secundária a paraganglioma, causando lesão medular completa. Foi realizado tratamento cirúrgico no Hospital Geral do Estado da Bahia, que apresentou sangramento excessivo como complicação que indicou necessidade de embolização prévia. Com isso, os autores recomendam cautela ao avaliar e tratar esta rara lesão causadora de déficit neurológico, cujo planejamento cirúrgico deve ser criterioso a fim de evitar complicações catastróficas.Paragangliomas are rare neuroendocrine tumors that originate in specialized cells derived from neural crest cells, affecting the region of the carotid or jugular-tympanic artery. They are rare within the spinal canal and, when they occur, they are normally found in the extramedullary intradural compartment of the lumbosacral region. This report presents the case of a 26-year-old male patient who was diagnosed with pathological fracture of the thoracic spine (T10, secondary to paraganglioma, thus causing complete spinal cord injury. Surgical treatment was performed at the General Hospital of the State of Bahia and the complication of excessive bleeding occurred, which indicated that there had been a need for prior embolization. Because of this, the authors recommend caution when evaluating and treating this rare lesion that causes neurological deficits, with careful surgical planning in order to catastrophic complications.

  7. Radiometric analysis of paraclinoid carotid artery aneurysms.

    Science.gov (United States)

    Tanaka, Yuichiro; Hongo, Kazuhiro; Tada, Tsuyoshi; Nagashima, Hisashi; Horiuchi, Tetsuyoshi; Goto, Tetsuya; Koyama, Jun-ichi; Kobayashi, Shigeaki

    2002-04-01

    Classification of paraclinoid carotid artery (CA) aneurysms based on their associated branching arteries has been confusing because superior hypophyseal arteries (SHAs) are too fine to appear opacified on cerebral angiograms. The authors performed a retrospective radiometric analysis of surgically treated paraclinoid aneurysms to elucidate their angiographic and anatomical characteristics. A retrospective analysis was made of 85 intradural paraclinoid aneurysms in which the presence or absence of branching arteries had been determined at the time of surgical clipping. The lesions were classified as supraclinoid, clinoid, and infraclinoid aneurysms based on their relation to the anterior clinoid process on lateral angiograms of the CA. The direction of the aneurysms were measured according to angles formed between the medial portion of the horizontal line crossing the aneurysm sac and the center of the aneurysm neck on anteroposterior angiograms. Branching arteries were associated with 68 aneurysms, of which 28 were ophthalmic artery (OphA) lesions (32.9%) and 40 were SHA ones (47.1%); associated branching arteries were absent in 17 aneurysms (20%). Twenty-five aneurysms (29.4%) were located at the supraclinoidal level, 46 (54.1%) at the clinoidal, and 14 (16.5%) at the infraclinoidal. The majority of aneurysms identified at the supraclinoidal level were OphA lesions (44%) or those unassociated with branching arteries (48%), with mean directions of 57 degrees or 67 degrees, respectively. At the clinoidal level, the mean directions of aneurysms were 76 degrees in six lesions unassociated with branching arteries (13%), 43 degrees in 16 OphA lesions (35%), and -11 degrees in 24 SHA ones (52%). All aneurysms at the infraclinoidal level arose at the origin of the SHAs, with a mean direction of -29 degrees, and most of these were embedded in the carotid cave. Aneurysms arising from the SHA can be distinguished from those not located at an arterial division by cerebral

  8. MR findings of medulloblastomas and the significance of contrast enhanced MR of brain and spine for the staging

    International Nuclear Information System (INIS)

    Chung, Jae Joon; Kim, Dong Ik; Chung, Tae Sub; Lee, Yeon Hee; Suh, Jung Ho

    1994-01-01

    The purpose of this study were to analyze the MR findings of medulloblastoma, and to evaluate the subarachnoid dissemination and the significance of contrast enhanced MR of brain and spine for tumor staging. The preoperative brain MR studies of 18 patients (9 males, 9 females; mean age, 9.4 years) with surgically proved medulloblastomas were retrospectively reviewed to characterize these neoplasms with regard to their location, size, MR signal intensity, appearance after contrast enhancement, presence of cyst and necrosis, subarachnoid dissemination, and other associated findings. In 14 patients postoperative spine MR studies were evaluated for staging and therapeutic planning. The most frequent location of medulloblastoma was the inferior vermis and the mean tumor size was 4.1 x 3.6 x 3.9 cm. On T1-weighted image, medulloblastomas generally had low to intermediate signal, predominantly hypointense relative to white matter. On T2-weighted image, medulloblastomas showed moderately high signal, hyperintense relative to white matter. Inhomogeneous contrast enhancement was demonstrated in 13 patients(72.2%) after injection of gadopentetate dimeglumine(Gadolinium). Cyst and necrosis within the tumor were visualized in 15 patients(83.3%). Subarachnoid disseminations of medulloblastomas were noted in 11 patients(61.1%), of which 6 demonstrated intracranial and 2 intraspinal dissemination. Three had both intracranial and intraspinal dissemination. In nine cases with intracranial lesions, there were intraparenchymal mass formation(7), subarachnoid nodules(5), infundibular lesions(2) and diffuse gyral enhancement(1). In five cases with intraspinal lesions, there were extramedullary intradural small nodules(3), central canal nodules(2), intradural masses(1) and fine nodular and sheet-like leptomeningeal enhancement(1). Other associated findings included intratumoral hemorrhage(11.1%), peritumoral edema(44.4%), tonsillar herniation(44.4%), hydrocephalus(88.9%) and

  9. Treatment of a persistent iatrogenic cerebrospinal fluid-pleural fistula with a cadaveric dural-pleural graft.

    Science.gov (United States)

    Raffa, Scott J; Benglis, David M; Levi, Allan D

    2009-04-01

    A cerebrospinal fluid (CSF)-pleural fistula is a unique condition with which all spine surgeons need to be familiar, particularly those who use anterolateral approaches to the thoracic region. When direct suturing of the dural defect is not possible, techniques for indirect repair must be considered. To report a novel technique for repair of a thoracic CSF-pleural fistula with a cadaveric dural-pleural graft after failure of initial treatment with Duragen (Integra, Plainsboro, NJ) and fibrin glue. Case report/University of Miami/Jackson Memorial Medical Center. The case of a 65-year-old female is presented. The patient represented 5 weeks after the initial operation (left-sided thoracotomy, partial T10-T11 corpectomy and removal of an intradural calcified disc) with dyspnea and severe positional headaches. Imaging of the brain revealed a subdural hematoma and magnetic resonance imaging of the thoracic region demonstrated a pseudomeningocele surrounding the postsurgical bed. She underwent a revision thoracotomy and CSF-pleural fistula repair. We addressed the dural opening indirectly by suturing a piece of cadaveric dural allograft to the thickened pleura, after Duragen and fibrin glue were placed over the dural defect. A lumbar drain and a chest tube were also placed. At 12 weeks post-op, the subdural hematoma had completely resolved and her chest X-rays demonstrated a significant decrease in the CSF-pleural collection. Anterolateral thoracic disc surgery poses a great challenge to the spine surgeon but can provide the most direct way of decompressing the spinal cord as a result of ventral pathology. Some of the most difficult aspects of dealing with a CSF leak in this area relates to 1) the relative complexity of suturing the dura directly as it is at a considerable distance from the operating surgeon; 2) the manner in which the contralateral dura slopes away and is hidden from view; and 3) the relatively negative intrathoracic pressure, which encourages the

  10. En Bloc Resection of Primary Malignant Bone Tumor in the Cervical Spine Based on 3-Dimensional Printing Technology.

    Science.gov (United States)

    Xiao, Jian-Ru; Huang, Wen-Ding; Yang, Xing-Hai; Yan, Wang-Jun; Song, Dian-Wen; Wei, Hai-Feng; Liu, Tie-Long; Wu, Zhi-Peng; Yang, Cheng

    2016-05-01

    To investigate the feasibility and safety of en bloc resection of cervical primary malignant bone tumors by a combined anterior and posterior approach based on a three-dimensional (3-D) printing model. Five patients with primary malignant bone tumors of the cervical spine underwent en bloc resection via a one-stage combined anteroposterior approach in our hospital from March 2013 to June 2014. They comprised three men and two women of mean age 47.2 years (range, 26-67 years). Three of the tumors were chondrosarcomas and two chordomas. Preoperative 3-D printing models were created by 3-D printing technology. Sagittal en bloc resections were planned based on these models and successfully performed. A 360° reconstruction was performed by spinal instrumentation in all cases. Surgical margins, perioperative complications, local control rate and survival rate were assessed. All patients underwent en bloc excision via a combined posterior and anterior approach in one stage. Mean operative time and estimated blood loss were 465 minutes and 1290 mL, respectively. Mean follow-up was 21 months. Wide surgical margins were achieved in two patients and marginal resection in three; these three patients underwent postoperative adjuvant radiation therapy. One vertebral artery was ligated and sacrificed in each of three patients. Nerve root involved by tumor was sacrificed in three patients with preoperative upper extremity weakness. One patient (Case 3) had significant transient radiculopathy with paresis postoperatively. Another (Case 4) with C 4 and C 5 chordoma had respiratory difficulties and pneumonia after surgery postoperatively. He recovered completely after 2 weeks' management with a tracheotomy tube and antibiotics in the intensive care unit. No cerebrovascular complications and wound infection were observed. No local recurrence or instrumentation failure were detected during follow-up. Though technically challenging, it is feasible and safe to perform en

  11. Somatostatin receptor imaging in intracranial tumours

    International Nuclear Information System (INIS)

    Schmidt, M.; Scheidhauer, K.; Voth, E.; Schicha, H.; Luyken, C.; Hildebrandt, G.; Klug, N.

    1998-01-01

    The somatostatin analogue [ 111 In-DTPA-d-Phe 1 ]-octreotide ( 111 In-octreotide) allows scintigraphic visualization of somatostatin receptor-expressing tissue. While it is well known that a large variety of tissues express somatostatin receptors and 111 In-octreotide scintigraphy has a clearly defined role in various neuroendocrine diseases, the clinical value of 111 In-octreotide scintigraphy in brain tumours is still under clinical investigation. In 124 patients with 141 brain lesions (63 meningiomas, 24 pituitary adenomas, 10 gliomas WHO class I and II, 12 gliomas WHO class III and IV, 11 neurinomas and 2 neurofibromas, 7 metastases and 12 other varieties: three non-Hodgkin B-cell lymphomas, two epidermoids, one abscess, one angioleiomyoma, one chordoma, one haemangiopericytoma, one osteosarcoma, one plasmacytoma and one pseudocyst), 111 In-octreotide scintigraphy was performed 4-6 and 24 h after i.v. injection of 110-220 MBq 111 In-octreotide. Planar images of the head in four views with a 128 x 128 matrix and single-photon emission tomographic images (64 x 64 matrix) were acquired, and lesions were graded according to qualitative tracer uptake. Fifty-nine of the 63 meningiomas showed moderate to intense tracer uptake. Nine of 24 pituitary adenomas were visible; the remaining 15 did not show any tracer uptake. None of the class I and II gliomas with an intact blood-brain barrier were detected whereas 11/12 class III and IV gliomas showed 111 In-octreotide uptake. None of the neurinomas or neurofibromas were positive. Five of seven metastases were classified as positive, as were the osteosarcoma, two of three non-Hodgkin B-cell lymphomas, one abscess, one angioleiomyoma, one chordoma and one haemangiopericytoma. The other varieties (one non-Hodgkin B-cell lymphoma, two epidermoids, one plasmacytoma and one pseudocyst) did not show 111 In-octreotide uptake. The results demonstrate that a large variety of intracranial lesions express somatostatin receptors and

  12. Particle Therapy Using Protons or Carbon Ions for Unresectable or Incompletely Resected Bone and Soft Tissue Sarcomas of the Pelvis

    Energy Technology Data Exchange (ETDEWEB)

    Demizu, Yusuke, E-mail: y_demizu@nifty.com [Department of Radiology, Hyogo Ion Beam Medical Center, Tatsuno, Hyogo (Japan); Jin, Dongcun; Sulaiman, Nor Shazrina; Nagano, Fumiko; Terashima, Kazuki; Tokumaru, Sunao [Department of Radiology, Hyogo Ion Beam Medical Center, Tatsuno, Hyogo (Japan); Akagi, Takashi [Department of Radiation Physics, Hyogo Ion Beam Medical Center, Tatsuno, Hyogo (Japan); Fujii, Osamu [Department of Radiation Oncology, Hakodate Goryokaku Hospital, Hakodate, Hokkaido (Japan); Daimon, Takashi [Department of Biostatistics, Hyogo College of Medicine, Nishinomiya, Hyogo (Japan); Sasaki, Ryohei [Division of Radiation Oncology, Kobe University Graduate School of Medicine, Kobe, Hyogo (Japan); Fuwa, Nobukazu [Department of Radiation Oncology, Ise Red Cross Hospital, Ise, Mie (Japan); Okimoto, Tomoaki [Department of Radiology, Hyogo Ion Beam Medical Center, Tatsuno, Hyogo (Japan)

    2017-06-01

    Purpose: To retrospectively analyze the treatment outcomes of particle therapy using protons or carbon ions for unresectable or incompletely resected bone and soft tissue sarcomas (BSTSs) of the pelvis. Methods and Materials: From May 2005 to December 2014, 91 patients with nonmetastatic histologically proven unresectable or incompletely resected pelvic BSTSs underwent particle therapy with curative intent. The particle therapy used protons (52 patients) or carbon ions (39 patients). All patients received a dose of 70.4 Gy (relative biologic effectiveness) in 32 fractions (55 patients) or 16 fractions (36 patients). Results: The median patient age was 67 years (range 18-87). The median planning target volume (PTV) was 455 cm{sup 3} (range 108-1984). The histologic type was chordoma in 53 patients, chondrosarcoma in 14, osteosarcoma in 10, malignant fibrous histiocytoma/undifferentiated pleomorphic sarcoma in 5, and other in 9 patients. Of the 91 patients, 82 had a primary tumor and 9 a recurrent tumor. The median follow-up period was 32 months (range 3-112). The 3-year rate of overall survival (OS), progression-free survival (PFS), and local control was 83%, 72%, and 92%, respectively. A Cox proportional hazards model revealed that chordoma histologic features and a PTV of ≤500 cm{sup 3} were significantly associated with better OS, and a primary tumor and PTV of ≤500 cm{sup 3} were significantly associated with better PFS. Ion type and number of fractions were not significantly associated with OS, PFS, or local control. Late grade ≥3 toxicities were observed in 23 patients. Compared with the 32-fraction protocol, the 16-fraction protocol was associated with significantly more frequent late grade ≥3 toxicities (18 of 36 vs 5 of 55; P<.001). Conclusions: Particle therapy using protons or carbon ions was effective for unresectable or incompletely resected pelvic BSTS, and the 32-fraction protocol was effective and relatively less toxic. Nevertheless, a

  13. Spinal meningiomas in dogs: 13 cases (1972-1987)

    International Nuclear Information System (INIS)

    Fingeroth, J.M.; Prata, R.G.; Patnaik, A.K.

    1987-01-01

    Medical records of 13 dogs with spinal meningiomas were reviewed. Breed predilections were not found. Males outnumbered females 9 to 4, and most of the dogs were middle-aged. All dogs had motor deficits of various degrees, and approximately half of the dogs had clinical signs of mild to moderate spinal pain. The remainder had histories of clinical signs suggestive of chronic discomfort. There was a prolonged (greater than 3 months) delay between the onset of signs and diagnosis, except in 3 dogs. The neurologic courses usually were progressive. Results of noncontrast spinal radiography were normal in 10 dogs; in 3 dogs, the lamina appeared scalloped. Results of myelography contributed to the correct diagnosis in 10 of 12 dogs; however, in 2 dogs, intradural/extramedullary tumors were thought to be intramedullary lesions. A preponderance of cervical meningiomas was found, accounting for 10 of 13 tumors. Lumbar meningiomas were found in the remaining 3 dogs. Surgery was performed in 9 of the dogs, six of which improved after surgery. Poor results were correlated with tumors that involved spinal cord segments of an intumescence, ventrally located tumors, iatrogenic trauma, and tumor invasion into adjacent neural parenchyma. Four of 13 spinal meningiomas were found to be invasive into the spinal cord itself

  14. Intracranial epidural hemorrhage during lumbar spinal surgery.

    Science.gov (United States)

    Imajo, Yasuaki; Kanchiku, Tsukasa; Suzuki, Hidenori; Yoshida, Yuichiro; Nishida, Norihiro; Goto, Hisaharu; Suzuki, Michiyasu; Taguchi, Toshihiko

    2016-01-01

    The authors report a case of intracranial epidural hemorrhage (ICEH) during spinal surgery. We could not find ICEH, though we recorded transcranial electrical stimulation motor evoked potentials (TcMEPs). A 35-year-old man was referred for left anterior thigh pain and low back pain that hindered sleep. Sagittal T2-weighted magnetic resonance imaging revealed an intradural tumor at L3-L4 vertebral level. We performed osteoplastic laminectomy and en bloc tumor resection. TcMEPs were intraoperatively recorded at the bilateral abductor digiti minimi (ADM), quadriceps, tibialis anterior and abductor hallucis. When we closed a surgical incision, we were able to record normal TcMEPs in all muscles. The patient did not fully wake up from the anesthesia. He had right-sided unilateral positive ankle clonus 15 min after surgery in spite of bilateral negative of ankle clonus preoperatively. Emergent brain computed tomography scans revealed left epidural hemorrhage. The hematoma was evacuated immediately via a partial craniotomy. There was no restriction of the patient's daily activities 22 months postoperatively. We should pay attention to clinical signs such as headache and neurological findgings such as DTR and ankle clonus for patients with durotomy and cerebrospinal fluid (CSF) leakage. Spine surgeons should know that it was difficult to detect ICEH by monitoring with TcMEPs.

  15. [Dumbbell malignant dorsal schwannoma embolized and operated by single posterior approach].

    Science.gov (United States)

    Zabalo, Gorka; de Frutos, Daniel; García, Juan Carlos; Ortega, Rodrigo; Guelbenzu, Juan José; Zazpe, Idoya

    2018-02-19

    We report a case of a 41 years old patient complaining of chronic dorsalgia. MRI showed a well defined intradural extramedular dumbbell-shaped lesion, associated to a left paravertebral tumor at D5-D6 level. The tumor was embolizated prior to surgery. Following she underwent a D4-D6 laminotomy, left D5-D6 costotransversectomy and resection of the intracanal extradural part of the lesion with section of the left D5 nerve. Posteriorly, complete resection of the extracanal portion of the tumor was performed by a postero-lateral approach. The anatomopathologic diagnosis was a malignant schwannoma. After the surgery, the patient required adyuvant radiotherapic treatment. Malignant schwannoma is a very uncommon tumor which belongs to the malignant peripheral nerve sheath tumors (MPNST). It develops from Schwann cells. It is frequently associated with type 1 neurofibromatosis. MPNST usually present local recurrence and can metastatize. Copyright © 2018 Sociedad Española de Neurocirugía. Publicado por Elsevier España, S.L.U. All rights reserved.

  16. Stereotactic radiosurgery for spinal metastases: a literature review

    International Nuclear Information System (INIS)

    Joaquim, Andrei Fernandes; Ghizoni, Enrico; Tedeschi, Helder; Pereira, Eduardo Baldon; Giacomini, Leonardo Abdala

    2013-01-01

    Objective: The spine is the most common location for bone metastases. Since cure is not possible, local control and relief of symptoms is the basis for treatment, which is grounded on the use of conventional radiotherapy. Recently, spinal radiosurgery has been proposed for the local control of spinal metastases, whether as primary or salvage treatment. Consequently, we carried out a literature review in order to analyze the indications, efficacy, and safety of radiosurgery in the treatment of spinal metastases. Methods: We have reviewed the literature using the PubMed gateway with data from the Medline library on studies related to the use of radiosurgery in treatment of bone metastases in spine. The studies were reviewed by all the authors and classified as to level of evidence, using the criterion defined by Wright. Results: The indications found for radiosurgery were primary control of epidural metastases (evidence level II), myeloma (level III), and metastases known to be poor responders to conventional radiotherapy – melanoma and renal cell carcinoma (level III). Spinal radiosurgery was also proposed for salvage treatment after conventional radiotherapy (level II). There is also some evidence as to the safety and efficacy of radiosurgery in cases of extramedullar and intramedullar intradural metastatic tumors (level III) and after spinal decompression and stabilization surgery. Conclusion: Radiosurgery can be used in primary or salvage treatment of spinal metastases, improving local disease control and patient symptoms. It should also be considered as initial treatment for radioresistant tumors, such as melanoma and renal cell carcinoma. (author)

  17. Imaging in spine and spinal cord malformations

    Energy Technology Data Exchange (ETDEWEB)

    Rossi, Andrea E-mail: a.rossi@panet.itandrearossi@ospedale-gaslini.ge.it; Biancheri, Roberta; Cama, Armando; Piatelli, Gianluca; Ravegnani, Marcello; Tortori-Donati, Paolo

    2004-05-01

    Spinal and spinal cord malformations are collectively named spinal dysraphisms. They arise from defects occurring in the early embryological stages of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal dysraphisms are categorized into open spinal dysraphisms (OSDs), in which there is exposure of abnormal nervous tissues through a skin defect, and closed spinal dysraphisms (CSD), in which there is a continuous skin coverage to the underlying malformation. Open spinal dysraphisms basically include myelomeningocele and other rare abnormalities such as myelocele and hemimyelo(meningo)cele. Closed spinal dysraphisms are further categorized based on the association with low-back subcutaneous masses. Closed spinal dysraphisms with mass are represented by lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category further comprises defects of midline notochordal integration (basically represented by diastematomyelia) and defects of segmental notochordal formation (represented by caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging (MRI) is the preferred modality for imaging these complex abnormalities. The use of the aforementioned classification scheme is greatly helpful to make the diagnosis.

  18. Natural history of cranial fibrous dysplasia revealed during long-term follow-up: Case report and literature review.

    Science.gov (United States)

    Penn, David L; Tartarini, Richard J; Glass, Carolyn H; De Girolami, Umberto; Zamani, Amir A; Dunn, Ian F

    2017-01-01

    Fibrous dysplasia (FD) is a rare developmental disease characterized by the replacement of bone marrow with proliferating fibro-osseous tissue. There exist three forms of FD-monostotic, polyostotic, and that associated with McCune-Albright syndrome. The disease can present in different locations and with a variety of symptoms. One of the more common locations of FD occurrence is the craniofacial region. Treatment of asymptomatic FD often involves conservative management with serial imaging. Medical management with bisphosphonates is an option, though long-term efficacy data are lacking. Surgical resection is usually reserved for very large or symptomatic lesions. We discuss the most unusual case of a 52-year-old male found to have a left pterional mass while being worked up for sinus headaches. The patient elected to follow this lesion conservatively, and imaging several years later showed obvious growth which accelerated in the last 4 years during an 18-year observational period. He ultimately underwent successful resection of an extradural and intradural FD. The significant growth potential of these lesions was revealed in this patient, in whom conservative management had been adopted. Despite optimal surgical resection and outcome in this case, the importance of surveillance imaging and perhaps earlier intervention cannot be underestimated when managing cranial FD.

  19. Thoracic disc herniation: Surgical treatment.

    Science.gov (United States)

    Court, C; Mansour, E; Bouthors, C

    2018-02-01

    Thoracic disc herniation is rare and mainly occurs between T8 and L1. The herniation is calcified in 40% of cases and is labeled as giant when it occupies more than 40% of the spinal canal. A surgical procedure is indicated when the patient has severe back pain, stubborn intercostal neuralgia or neurological deficits. Selection of the surgical approach is essential. Mid-line calcified hernias are approached from a transthoracic incision, while lateralized soft hernias can be approached from a posterolateral incision. The complication rate for transthoracic approaches is higher than that of posterolateral approaches; however, the former are performed in more complex herniation cases. The thoracoscopic approach is less invasive but has a lengthy learning curve. Retropleural mini-thoracotomy is a potential compromise solution. Fusion is recommended in cases of multilevel herniation, herniation in the context of Scheuermann's disease, when more than 50% bone is resected from the vertebral body, in patients with preoperative back pain or herniation at the thoracolumbar junction. Along with complications specific to the surgical approach, the surgical risks are neurological worsening, dural breach and subarachnoid-pleural fistulas. Giant calcified herniated discs are the largest contributor to myelopathy, intradural extension and postoperative complications. Some of the technical means that can be used to prevent complications are explored, along with how to address these complications. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  20. Melanotic Schwannomas Are Rarely Seen Pigmented Tumors with Unpredictable Prognosis and Challenging Diagnosis.

    Science.gov (United States)

    Keskin, Elif; Ekmekci, Sumeyye; Oztekin, Ozgur; Diniz, Gulden

    2017-01-01

    Melanotic Schwannoma (MS) is rarely seen and potentially malignant neoplasm that is categorized as a variant of Schwannoma. MS most frequently involves intracranial structures followed by posterior nerve roots in the spinal canal. Approximately 50% of the cases with MS have psammomatous calcifications and this type of MS is related to Carney complex with autosomal dominant inheritance. Most cases of MS are benign, though 10% of them are malignant with metastatic potential. MS mimics melanoma and the differential diagnosis should be made excluding other melanin producing neoplasms especially melanoma. Case 1 . A 42-year-old hypertensive male presented for checkup. He had a well-defined extraspinal oval lesion measuring 3.5 × 2.5 cm near right adrenal. Case 2 . A 22-year-old female presented with neurofibromatosis-2, bilateral acoustic schwannomas and café au lait lesions on sacrococcygeal region. She had an intradural extramedullary lesion measuring 6.1 × 2.0 cm at L1-2 level. MS is a rare neoplasm composed of Schwann cells and melanin pigment. These tumors are usually benign but they may become aggressive. The biologic behavior of MS is difficult to predict; the patients have to be followed up for a longer period due to its malignant potential.

  1. Acute non-traumatic idiopathic spinal subdural hematoma: radiographic findings and surgical results with a literature review.

    Science.gov (United States)

    Kobayashi, Kazuyoshi; Imagama, Shiro; Ando, Kei; Nishida, Yoshihiro; Ishiguro, Naoki

    2017-11-01

    Intraspinal hematoma is a serious condition, and early diagnosis is necessary to permit emergency treatment. Among such hematomas, non-traumatic spinal subdural hematoma is a rare occurrence. We have experienced three patients with surgically proven subdural spinal hematoma, and here we report these cases with a review of their clinical and imaging characteristics. All three cases were idiopathic with no history of disease, no coagulopathy, and no trauma. All had acute onset that brought about paralysis of the lower limbs with severe pain. Early surgery was performed, based on a relatively early diagnosis using thoracolumbar MRI and CT. Since the epidural fat is not affected by bleeding, the normal structure remains and the boundary between hematoma and fat is a significant feature in MRI and CT. Partial Gd enhancement in MRI and ring enhancement in contrast CT were also apparent. Two of the cases had subarachnoid hematomas. Preoperative diagnosis of spinal subarachnoid hematoma is difficult because there are no specific radiological findings and confirmation can only occur intraoperatively. In particular, one case had a massive hematoma causing canal stenosis, and it was difficult to distinguish between intradural and extradural hematoma. In all cases of subarachnoid or subdural hematoma, decompression was performed within 24 h after onset, and consequently, the patients had relatively good outcomes.

  2. Efficacy of Conical Laminoplasty After Thoracic Laminectomy.

    Science.gov (United States)

    Kobayashi, Kazuyoshi; Imagama, Shiro; Ando, Kei; Nishida, Yoshihiro; Ishiguro, Naoki

    2017-07-07

    Retrospective clinical study. To evaluate the results of a new method of laminoplasty, referred to as conical laminoplasty. In surgery for a thoracic intradural tumor, laminectomy is frequently used for tumor resection. However, laminoplasty may be more effective due to prevention of damage to the posterior elements. This method can be performed as recapping T-saw laminoplasty and conical laminoplasty which we refer. After en bloc laminectomy, conical laminoplasty (group C; 21 cases) or recapping T-saw laminoplasty (group R; 17 cases) was performed for patients with thoracic spinal cord tumors. If neither was possible, laminectomy (group L; 16 cases) was performed. The number of laminae removed and Japanese Orthopedic Association recovery rates did not differ significantly among the 3 groups. Sagittal kyphosis significantly worsened at final follow-up in group L, but did not change significantly in groups C and R. Conical laminoplasty is particularly useful in a case in which T-saw laminoplasty cannot be performed due to tumor expansion in the canal and ossification of the ligamentum flavum. Conical laminoplasty also preserves the posterior elements, which reduces the risk of worsening of kyphosis postoperatively.

  3. Pyogenic and non-pyogenic spinal infections: emphasis on diffusion-weighted imaging for the detection of abscesses and pus collections

    Science.gov (United States)

    Kim, J; Capizzano, A A; Kirby, P; Kademian, J; Sato, Y

    2014-01-01

    The incidence of spinal infections has increased in the past two decades, owing to the increasing number of elderly patients, immunocompromised conditions, spinal surgery and instrumentation, vascular access and intravenous drug use. Conventional MRI is the gold standard for diagnostic imaging; however, there are still a significant number of misdiagnosed cases. Diffusion-weighted imaging (DWI) with a b-value of 1000 and apparent diffusion coefficient (ADC) maps provide early and accurate detection of abscess and pus collection. Pyogenic infections are classified into four types of extension based on MRI and DWI findings: (1) epidural/paraspinal abscess with spondylodiscitis, (2) epidural/paraspinal abscess with facet joint infection, (3) epidural/paraspinal abscess without concomitant spondylodiscitis or facet joint infection and (4) intradural abscess (subdural abscess, purulent meningitis and spinal cord abscess). DWI easily detects abscesses and demonstrates the extension, multiplicity and remote disseminated infection. DWI is often a key image in the differential diagnosis. Important differential diagnoses include epidural, subdural or subarachnoid haemorrhage, cerebrospinal fluid leak, disc herniation, synovial cyst, granulation tissue, intra- or extradural tumour and post-surgical fluid collections. DWI and the ADC values are affected by susceptibility artefacts, incomplete fat suppression and volume-averaging artefacts. Recognition of artefacts is essential when interpreting DWI of spinal and paraspinal infections. DWI is not only useful for the diagnosis but also for the treatment planning of pyogenic and non-pyogenic spinal infections. PMID:24999081

  4. Melanotic schwannoma of the lumbar spine: a case report and literature review

    Directory of Open Access Journals (Sweden)

    João Bernardo Sancio Rocha Rodrigues

    2015-06-01

    Full Text Available Schwannomas are benign tumors, usually solitary, encapsulated, slow-growing, which have their origin in differentiated neoplastic Schwann cells with extramedullary intradural usual development related to nerve roots. The melanotic schwannoma is a variant of these tumors whose location in almost one third of cases is on the posterior spinal nerve root, with a nonspecific clinical presentation. Magnetic resonance imaging is the most widely used test for the diagnosis, revealing hyperintense T1-weighted sequences and hypointense T2-weighted sequences. Diagnostic confirmation is obtained by histological and immunohistochemical studies, in which there is intense cytoplasmatic pigmentation. There are two distinct types of melanotic schwannomas: sporadic and psammomatous, the latter related to the called Carney complex, a form of multiple endocrine neoplasm with familiar character. In literature we found few cases of these neoplasms, the largest series consisting of five cases. The objective of this study is to report a rare case of melanotic schwannoma of the lumbar spine of the sporadic type of extramedullary location. We also present a brief review of the literature containing the main characteristics of the tumor, including its different forms, differential diagnoses, data from histological and immunohistochemical studies as well as the currently recommended approach in order to contribute to a better understanding of this neoplasm.

  5. Intramedullary spinal cord metastasis from prostate carcinoma: a case report

    Directory of Open Access Journals (Sweden)

    Lieberson Robert E

    2012-06-01

    Full Text Available Abstract Introduction Although vertebral and epidural metastases are common, intradural metastases and intramedullary spinal cord metastases are rare. The indications for the treatment of intramedullary spinal cord metastases remain controversial. We present the first biopsy-proven case of an intramedullary spinal cord metastasis from adenocarcinoma of the prostate. Case presentation Our patient was a 68-year-old right-handed Caucasian man with a Gleason grade 4 + 3 prostate adenocarcinoma who had previously undergone a prostatectomy, androgen blockade and transurethral debulking. He presented with new-onset saddle anesthesia and fecal incontinence. Magnetic resonance imaging demonstrated a spindle-shaped intramedullary lesion of the conus medullaris. Our patient underwent decompression and an excisional biopsy; the lesion’s pathology was consistent with metastatic adenocarcinoma of the prostate. Postoperatively, our patient received CyberKnife® radiosurgery to the resection cavity at a marginal dose of 27Gy to the 85% isodose line. At three months follow-up, our patient remains neurologically stable with no new deficits or lesions. Conclusions We review the literature and discuss the indications for surgery and radiosurgery for intramedullary spinal cord metastases. We also report the novel use of stereotactic radiosurgery to sterilize the resection cavity following an excisional biopsy of the metastasis.

  6. Relationship Between Cervical Disc Herniation and Posterior Longitudinal Ligament in Intra-operative Microscope-Guided 468 ACDF

    Directory of Open Access Journals (Sweden)

    LI Ze-qing

    2016-12-01

    Full Text Available Objective: To observe the relationship between cervical disc herniation (CDH and posterior longitudinal ligament (PLL under microscope-guided anterior cervical decompression and fusion (ACFD, with intention to provide the basis for PLL surgery resection.Methods: Totally 325 patients with CDH (468 levels were performed with microscope-guided ACFD. During the procedure, integrity of annulus, PLL, and dural sac were observed. Meanwhile, the position of the protruded mass was recorded. The actual exploratory results under microscope were documented and compared with preoperative magnetic resonance imaging (MRI presentation.Results: According to disc-posterior anatomic layers and findings under the intraoperative microscope, 468 levels of CDH were classified into five sites. Site I: disc with tearing annulus but intact outmost layer (in 47 levels; Site Ⅱ: ruptured annulus with intact PLL (in 202 levels;Site Ⅲ: herniated mass within or through PLL (in 107 levels; Site Ⅳ: free and sequestered mass posterior to PLL (in 105 levels; Site V: intradural CDH (in 7 levels.Conclusion: This observation of CDH reveals the chronic and dynamic progress of the disease. Though MRI is considered a powerful and sensitive technique for the assessment of CDH, it may not provide the proof for the relationship between CDH and PLL. To ensure the complete anterior decompression, the PLL should be resected routinely to explore if there is free disc mass under PLL.

  7. Is there a causal relationship between the hypoxia-ischaemia associated with cardiorespiratory arrest and subdural haematomas? An observational study.

    Science.gov (United States)

    Hurley, M; Dineen, R; Padfield, C J H; Wilson, S; Stephenson, T; Vyas, H; McConachie, N; Jaspan, T

    2010-09-01

    The aim of this study was to determine the frequency of subdural haematomas (SDHs) occurring in infants presenting following atraumatic cardiorespiratory collapse. This study was a review of retrospective case notes, brain imaging and post-mortem examinations carried out in the paediatric intensive care unit (PICU) and emergency department (ED) in a tertiary paediatric centre in the UK. The study included infants and children less than 4 years old dying in the ED or admitted to the PICU after atraumatic cardiorespiratory arrest. We identified macroscopic SDHs on brain imaging or post-mortem examination. Of those children who experienced a cardiorespiratory arrest from a non-traumatic cause and met inclusion criteria, 33 presented and died in the ED and 17 were admitted to the PICU. These children had a post-mortem examination, brain imaging or both. None of these infants had a significant SDH. One child had a small clot adherent to the dura found on post-mortem and two had microscopic intradural haemorrhage, but it is unclear in each case whether this was artefact, as each had otherwise normal brains. Subdural haematoma arising in infants or young children in the context of catastrophic cardiorespiratory compromise from a non-traumatic cause was not observed.

  8. Is there a causal relationship between the hypoxia–ischaemia associated with cardiorespiratory arrest and subdural haematomas? An observational study

    Science.gov (United States)

    Hurley, M; Dineen, R; Padfield, C J H; Wilson, S; Stephenson, T; Vyas, H; Mcconachie, N; Jaspan, T

    2010-01-01

    The aim of this study was to determine the frequency of subdural haematomas (SDHs) occurring in infants presenting following atraumatic cardiorespiratory collapse. This study was a review of retrospective case notes, brain imaging and post-mortem examinations carried out in the paediatric intensive care unit (PICU) and emergency department (ED) in a tertiary paediatric centre in the UK. The study included infants and children less than 4 years old dying in the ED or admitted to the PICU after atraumatic cardiorespiratory arrest. We identified macroscopic SDHs on brain imaging or post-mortem examination. Of those children who experienced a cardiorespiratory arrest from a non-traumatic cause and met inclusion criteria, 33 presented and died in the ED and 17 were admitted to the PICU. These children had a post-mortem examination, brain imaging or both. None of these infants had a significant SDH. One child had a small clot adherent to the dura found on post-mortem and two had microscopic intradural haemorrhage, but it is unclear in each case whether this was artefact, as each had otherwise normal brains. Subdural haematoma arising in infants or young children in the context of catastrophic cardiorespiratory compromise from a non-traumatic cause was not observed. PMID:20647510

  9. 20 year old lady with a paraspinal mass.

    LENUS (Irish Health Repository)

    O'Toole, Orna

    2010-05-01

    A 20 year old female presented with a 4 month history of right upper limb pain and paraesthesias. She had no systemic symptoms and no prior medical or family history of note. MRI revealed a right-sided intradural extramedullary mass extending from C7-T1 and displacing the spinal cord. While awaiting surgery her symptoms progressed to involve the right lower limb. She was re-imaged and the lesion now extended from C5 to T3 with spinal cord compression at C7-T1. The radiological features and recent rapid growth were felt to be in keeping with a large plexiform neurofibroma. The patient underwent emergency resection of the lesion and pathology revealed Hodgkin\\'s Lymphoma (HL)-mixed cellularity type. A mediastinal mass was identified on further imaging and biopsy confirmed the diagnosis of HL-stage IV. The patient is currently undergoing treatment with ABVD chemotherapy. CNS-HL is extremely rare and may occur de novo or in association with systemic disease. Lesions may be parenchymal or dural based and are usually intracranial with an increased risk of CNS involvement in HL-mixed-cellularity type as in our patient. This is the first report in the literature of CNS-HL radiologically mimicking a paraspinal plexiform neurofibroma.

  10. Fuji computed radiography (FCR) for the diagnosis of spinal disorders

    International Nuclear Information System (INIS)

    Kubo, Yoshichika; Waga, Shiro; Kojima, Tadashi

    1987-01-01

    Since April, 1985, we have, in co-operation with the Fuji Film Co., Ltd., used Fuji Computed Radiography (FCR) in the diagnosis of spinal disorders. FCR is a new computed radiographic system which uses an energy-storage phosphorus panel called an ''Imaging Plate'' as an image sensor. The ''Imaging Plate'' can be used to obtain radiographs in exactly the same way as the screen-film combination used in conventional radiography; X-rays are exposed on the ''Imaging Plate'' instead of X-ray film in the conventional fashion, and then the ''Imaging Plate'' is calculated. The processed digital data from the scans is transformed into a picture by means of digital-to-analogue conversion. The pictures are always clear and beautiful. Plain films of the spine taken by FCR are even clearer, even in the cervicothoracic region, where it is usually difficult to obtain clear cervicothoracic films in conventional radiography. We can obtain much precise information about the spinal posterior osteophytes and grafted bone, even when the patients are immobilized in a Halo apparatus. In myelography, the pictures are also clearer; we can see how the root or roots are compressed, how the dura and cord are involved in patients with cervical disc disease, and even the very narrow space between the cord and an intradural tumor. FCR is thus very useful in observing spinal disorders, either in plain films or myelography. (author)

  11. Primary intraosseous meningiomas

    International Nuclear Information System (INIS)

    Arana, E.; Diaz, C.; Latorre, F.F.; Menor, F.; Revert, A.; Beltran, A.; Navarro, M.

    1996-01-01

    Purpose: A retrospective study was performed to evaluate the clinical, radiological and histological findings of 14 intraosseous meningiomas. Material and Methods: 14 histologically proved intraosseous meningiomas were studied with plain skull film and CT. Pathological records were reviewed. Results: We found 9 calvarial cases and 5 spheno-orbital ones. The most common symptom in the calvarial cases was a lump in the scalp, and in the spheno-orbital lesions it was exophtalmos. Hyperostosis was present in all 5 spheno-orbital cases and in 3 out of the 9 calvarial ones; in the other 6 cases it had a mixed pattern. Enhanced dura was present in 3 calvarial cases and in 2 sphenoidal ones. Dura was involved in 5 cases: 2 with inflammatory changes, 2 with a minimal intradural tumour and one with a subdural tumour. Conclusion: CT better detected both the bony reaction and the intraosseous extension of the tumour. The dural changes were not specific of tumoural involvement. The differential diagnosis comprises: fibrous dysplasia, osteomas, blastic metastasis and mainly meningioma en plaque (MEP). Comparing our findings with other series, we noticed that in some cases the term MEP was used for similar cases. As the name MEP is merely descriptive and both entities share a larger number of similarities than differences, we believe that a differentiation between hyperostotic en plaque meningioma and intraosseous meningioma can hardly be made. (orig.)

  12. Intracranial repair of posttraumatic cerebrospinal fluid rhinorrhea associated with recurrent meningitis.

    Science.gov (United States)

    Yaldiz, Can; Ozdemir, Nail; Yaman, Onur; Seyin, İsmail Ertan; Oguzoglu, Serdar

    2015-01-01

    The purposes of this study are to assess the efficacy of our intracranial surgery and evaluate the association between failure after first surgical repair and the risk factors that have been applied on a group of 13 patients affected by posttraumatic cerebrospinal fluid rhinorrhea associated with recurrent meningitis. We retrospectively collected data on 13 patients referred to our institution. All patients had history of head trauma and experienced 2 or more episodes of meningitis. Three of the 13 patients had craniectomy defect due to previous trauma and surgery, 9 patients had linear fracture, and 1 patient had no apparent fracture line on preoperative radiologic evaluation. Ten of the 13 patients had identified frontal bone fracture involving the frontal sinus during surgery. Dural tear was identified intradurally and was repaired using a fascia lata graft with or without fibrin glue. Fibrin glue was applied over the suture in 7 patients. Three of the 13 patients had large dural defects. The size of bone and dural defect seems to be an important prognostic factor of episodes of meningitis. The use of fibrin glue to fixate fascia lata graft did not benefit the outcome.

  13. Stereotactic radiosurgery for spinal metastases: a literature review; Radiocirurgia estereotaxica para metastases de coluna vertebral: revisao de literatura

    Energy Technology Data Exchange (ETDEWEB)

    Joaquim, Andrei Fernandes; Ghizoni, Enrico; Tedeschi, Helder; Pereira, Eduardo Baldon; Giacomini, Leonardo Abdala, E-mail: andjoaquim@yahoo.com [Universidade Estadual de Campinas (UNICAMP), Campinas, SP (Brazil)

    2013-04-15

    Objective: The spine is the most common location for bone metastases. Since cure is not possible, local control and relief of symptoms is the basis for treatment, which is grounded on the use of conventional radiotherapy. Recently, spinal radiosurgery has been proposed for the local control of spinal metastases, whether as primary or salvage treatment. Consequently, we carried out a literature review in order to analyze the indications, efficacy, and safety of radiosurgery in the treatment of spinal metastases. Methods: We have reviewed the literature using the PubMed gateway with data from the Medline library on studies related to the use of radiosurgery in treatment of bone metastases in spine. The studies were reviewed by all the authors and classified as to level of evidence, using the criterion defined by Wright. Results: The indications found for radiosurgery were primary control of epidural metastases (evidence level II), myeloma (level III), and metastases known to be poor responders to conventional radiotherapy - melanoma and renal cell carcinoma (level III). Spinal radiosurgery was also proposed for salvage treatment after conventional radiotherapy (level II). There is also some evidence as to the safety and efficacy of radiosurgery in cases of extramedullar and intramedullar intradural metastatic tumors (level III) and after spinal decompression and stabilization surgery. Conclusion: Radiosurgery can be used in primary or salvage treatment of spinal metastases, improving local disease control and patient symptoms. It should also be considered as initial treatment for radioresistant tumors, such as melanoma and renal cell carcinoma. (author)

  14. Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies.

    Science.gov (United States)

    Zanoletti, E; Girasoli, L; Borsetto, D; Opocher, G; Mazzoni, A; Martini, A

    2017-10-01

    Endolymphatic sac tumour (ELST) is infrequent, as emerges from small series reported in the literature. It is a slow-growing malignancy with local aggressiveness and a low risk of distant metastases. It is often misdiagnosed because of the late onset of symptoms and difficulty in obtaining a biopsy. Its frequency is higher in von Hippel-Lindau (VHL) disease (a genetic systemic syndrome involving multiple tumours), with a prevalence of around 25%. The diagnosis is based on radiology, with specific patterns on contrast-enhanced MRI and typical petrous bone erosion on bone CT scan. Our experience of ELST in the years between 2012-2015 concerns 7 cases, one of which was bilateral, in patients with VHL disease. Four of the 7 patients underwent 5 surgical procedures at our institution. Each case is described in detail, including clinical symptoms, and the intervals between symptom onset, diagnosis and therapy. Postoperative morbidity was low after early surgery on small tumours, whereas extensive surgery for large tumours was associated with loss of cranial nerve function (especially VII, IX, X). The critical sites coinciding with loss of neurological function were the fallopian canal, jugular foramen, petrous apex and intradural extension into the posterior cranial fossa. Early surgery on small ELST is advocated for patients with VHL disease, in whom screening enables a prompt diagnosis and consequently good prognosis. © Copyright by Società Italiana di Otorinolaringologia e Chirurgia Cervico-Facciale, Rome, Italy.

  15. Huge interparietal posterior fontanel meningohydroencephalocele

    Directory of Open Access Journals (Sweden)

    Jorge Félix Companioni Rosildo

    2015-03-01

    Full Text Available Congenital encephalocele is a neural tube defect characterized by a sac-like protrusion of the brain, meninges, and other intracranial structures through the skull, which is caused by an embryonic development abnormality. The most common location is at the occipital bone, and its incidence varies according to different world regions. We report a case of an 1-month and 7-day-old male child with a huge interparietal-posterior fontanel meningohydroencephalocele, a rare occurrence. Physical examination and volumetric computed tomography were diagnostic. The encephalocele was surgically resected. Intradural and extradural approaches were performed; the bone defect was not primarily closed. Two days after surgery, the patient developed hydrocephaly requiring ventriculoperitoneal shunting. The surgical treatment of the meningohydroencephalocele of the interparietal-posterior fontanel may be accompanied by technical challenges and followed by complications due to the presence of large blood vessels under the overlying skin. In these cases, huge sacs herniate through large bone defects including meninges, brain, and blood vessels. The latter present communication with the superior sagittal sinus and ventricular system. A favorable surgical outcome generally follows an accurate strategy taking into account individual features of the lesion.

  16. Long Segment Spinal Dural Cyst: A Case Report.

    Science.gov (United States)

    Aoyama, Tatsuro; Miyaoka, Yoshinari; Ogiwara, Toshihiro; Ito, Kiyoshi; Seguchi, Tatsuya; Hongo, Kazuhiro

    2016-04-01

    Spinal meningeal cysts are a rare benign disease that can cause myelopathy. In most cases, spinal meningeal cysts consist of an arachnoid membrane. To the best of our knowledge, few articles have report on intradural spinal meningeal cyst consisting of dura mater. A 58-year-old man presented to our institute with aggravation of clumsy hands and dysesthesia of the feet. Magnetic resonance imaging of the entire spine revealed a cystic lesion compressing the spinal cord posteriorly. Cyst fenestration and placement of the cyst-subarachnoid shunt was performed via an anterior approach. Postoperatively, the histopathologic results revealed that the cyst wall consisted of a dura mater-like membrane. The patient's symptoms resolved without the appearance of any new neurologic deficits. The etiology of spinal meningeal cysts remains unclear. Spinal meningeal cysts consisting of dura mater (spinal dural cysts) are extremely rare. Treatment with only decompression with laminectomy causes enlargement of the dural cyst later. Cyst fenestration and placement of a cyst-subarachnoid shunt for the spinal dural cyst resulted in the resolution of myelopathy and cyst shrinkage. Copyright © 2016 Elsevier Inc. All rights reserved.

  17. Posterior Laminoplastic Laminotomy Combined with a Paraspinal Transmuscular Approach for Removing a Lumbar Dumbbell-shaped Schwannoma: A Technical Note.

    Science.gov (United States)

    Ngerageza, Japhet Gideon; Ito, Kiyoshi; Aoyama, Tatsuro; Murata, Takahiro; Horiuchi, Tetsuyoshi; Hongo, Kazuhiro

    2015-01-01

    The surgical strategies and methods used to treat dumbbell-shaped tumors located in the lumbar-foraminal region are controversial. Although a total facetectomy and combined intra- and extraspinal canal approach provide a wide operative field, facet fusion is required, which can be rather invasive. Here, we report a successful removal of a lumbar dumbbell-shaped schwannoma using a combined laminoplastic laminotomy with Wiltse's paraspinal surgical approach. This was performed under an operating microscope without a complete facetectomy, fusion, and posterior fixation. Briefly, we treated two patients with lumbar foraminal tumors, both dumbbell-shaped schwannomas located in the intra- and extradural portion. After a laminoplastic laminotomy, the intradural tumor was removed. The tumor located at the extracanalicular site was removed after drilling the pars interarticularis of the lamina, which was performed to enlarge the intervertebral foramen via Wiltse's paraspinal surgical approach. During surgery, facetectomy with posterior fixation was not needed to remove the intraforaminal component. There was no lumbar instability or complication after surgery. Our results suggest that a combined posterior laminoplastic laminotomy and Wiltse's paraspinal surgical approach is useful and less invasive for treating patients with lumbar foraminal tumors.

  18. Improved magnetic resonance myelography using image fusion

    International Nuclear Information System (INIS)

    Eberhardt, K.; Ganslandt, O.; Stadlbauer, A.; Landesklinikum St. Poelten

    2013-01-01

    To demonstrate that the disadvantage of missing anatomical information in heavily T2-weighted MR myelography images can be eliminated by image fusion and phase encoding in the coronal direction of the source images, resulting in MR myelography images comparable to the gold standard, i. e., post-myelography CT. This study included 110 patients suffering from extradural pathologies of the cervical and lumbar spine. All patients were investigated using 3D MR myelography and post-myelography CT. The MRI data were post-processed using image fusion and reconstruction algorithms and were compared to the corresponding images of post-myelography CT. Our approach for visualization (3D MR myelography) was able to depict intradural structures in high spatial resolution and without artifacts. The results of our visualization approach were comparable to the gold standard - post-myelography CT. Anatomical correlation was reached by image fusion of different MR data sets. The required post-processing steps were performed quickly and were available on a commercial workstation. Image fusion of different MR data sets allows for visualization of 3D data sets with enhanced quality. The results for the visualization of MR myelography in particular are comparable to conventional myelography and post-myelography CT. The missing anatomical information in heavily T2-weighted MR myelography images can be compensated by image fusion with conventional MRI. (orig.)

  19. Neurofibromatosis Type 2 Presenting with Oculomotor Ophthalmoplegia and Distal Myopathy

    Directory of Open Access Journals (Sweden)

    Jessica Mani Penny Tevaraj

    2016-01-01

    Full Text Available Neurofibromatosis type 2 usually presents with bilateral acoustic schwannomas. We highlight the rare presentation of neurofibromatosis initially involving third nerve. A 23-year-old Malay female presented with left eye drooping of the upper lid and limitation of upward movement for 8 years. It was associated with right-sided body weakness, change in voice, and hearing disturbance in the right ear for the past 2 years. On examination, there was mild ptosis and limitation of movement superiorly in the left eye. Both eyes had posterior subcapsular cataract. Fundoscopy showed generalised optic disc swelling in both eyes. She also had palsy of the right vocal cord, as well as the third and eighth nerve. There was wasting of the distal muscles of her right hand, with right-sided decreased muscle power. Pedunculated cutaneous lesions were noted over her body and scalp. MRI revealed bilateral acoustic and trigeminal schwannomas with multiple extra-axial lesions and intradural extramedullary nodules. Patient was diagnosed with neurofibromatosis type 2 and planned for craniotomy and tumour debulking, but she declined treatment. Neurofibromatosis type 2 may uncommonly present with isolated ophthalmoplegia, so a thorough physical examination and a high index of suspicion are required to avoid missing this condition.

  20. Rare case of pneumorrhachis, pneumomediastinum, pneumothorax, and surgical emphysema secondary to bronchial asthma

    Directory of Open Access Journals (Sweden)

    Mahajan PS

    2014-03-01

    Full Text Available Parag Suresh Mahajan,1 Nasser Jassim Al Maslamani,1 Nishan K Purayil2 1Department of Radiology, 2Accident and Emergency Department, Al Khor Hospital, Hamad Medical Corporation, Doha, Qatar Abstract: Air localized within the spinal canal is called pneumorrhachis. In the case of pneumorrhachis, air can be present in the extradural, the intradural, or the subarachnoid space of the spinal canal. The air within the soft tissue of the posterior mediastinum may dissect along fascial planes, through the intervertebral neural foramina, and into the extradural or the subarachnoid space. Nontraumatic pneumorrhachis is a rare presentation. Most of the time, pneumorrhachis is asymptomatic, remains localized, and resolves spontaneously. There are very few reports of combined presence of pneumomediastinum and extradural pneumorrhachis not associated with thoracic injury in the published literature. We report a rare case of pneumorrhachis, pneumomediastinum, pneumothorax, and surgical emphysema in an adult female patient developed after a bout of violent cough related to bronchial asthma. Keywords: pneumorrhachis, pneumomediastinum, pneumothorax, surgical emphysema, asthma

  1. Intraspinal dural-based primary osteoblastoma with aneurysmal bone cyst-like change.

    Science.gov (United States)

    Fu, Xinge; Jiang, Juhong; Luo, Bo-ning; Tian, Xiao-ying; Li, Zhi

    2014-10-01

    Osteoblastoma is a benign bone-forming neoplasm that occurs commonly in the posterior elements of the spine and the sacrum. However, so far there has been no report of intradural osteoblastoma described in the literature. We present a unique case of intraspinal dural-based osteoblastoma with aneurysmal bone cyst-like change without evidence of vertebral involvement. An 11-year-old Chinese girl presented with a 3-month history of gradually progressive back pain and a weakness of both lower limbs. Thoracic MRI revealed a well-demarcated subdural mass at the T5 level with heterogeneous enhancement. Histologically, the tumor was found to be attached to the dura and composed of numerous osteoid spicules and trabecular bone with diffusely scattered osteoclast-type, multinucleated giant cells. Ectactic blood vessels and blood-filled cystic spaces were also observed. A diagnosis of primary intraspinal dural-based osteoblastoma with aneurysmal bone cyst-like change was made. To our best knowledge, this is possibly the first case of primary osteoblastoma arising from meninges. Meningeal osteocartilaginous tumors are rare, with obscure histogenesis. The differential diagnosis of osteoblastoma in unusual locations is difficult and the confirmation of diagnosis should be cautiously made. Awareness of dural-based osteoblastoma and its histological features is important to avoid a diagnostic pitfall caused by histological similarities to other intra-craniospinal lesions with osteoid differentiation or bone formation. © 2014 Japanese Society of Neuropathology.

  2. [Glomus jugulare tumor: perioperative management].

    Science.gov (United States)

    Ferrando, A; Fraile, J R; Bermejo, L; de Miguel, A; Aristegui, M; Hervías, M; Quirós, P

    1996-12-01

    Surgical treatment of glomus jugulare tumors yields high rates of perioperative morbidity and mortality for several reasons, among them neuroendocrine secretory activity, a high degree of vascularization, intracranial extension, duration of surgery and cranial nerve lesion. Secretory activity (e.g. catecholamines and serotonin) should be investigated before surgery and treated appropriately. Carotid arteriography (and ball occlusion) are useful to assess vascularization of the tumor and determine the need to clamp the carotid artery during the procedure. Potential complications such as hemodynamic alterations (bleeding or endocrine response), pulmonary embolism (air or thrombotic), hypothermia, facial nerve lesion, should be monitored for during surgery. After surgery cranial nerve involvement, which can lead to dysphagia and bronchoaspiration, must be looked for; the risk of cerebro-spinal fluid fistula is also high. We report the case of a woman who underwent surgery for a non secreting glomus jugulare tumor with extradural intracranial invasion. The main complications during surgery were bleeding with hemodynamic repercussions, pulmonary embolism, lesions in the VII, VIII and X cranial nerves, and opening of the dura mater (which required insertion of an intradural drain to prevent formation of a fistula). After surgery oral intake was delayed until intestinal function was established and glottic sphincter competence was verified by fiberoptic laryngoscopy. The only complication presenting at this time was cephalea, which disappeared upon removal of the drain on day 4. The patient was released on day 10.

  3. Retrograde intraventricular hemorrhage caused by a traumatic sacral pseudomeningocele in the presence of spina bifida occulta. Case report.

    Science.gov (United States)

    Uff, Christopher; Bradford, Robert

    2005-11-01

    The authors present the case of a large, posttraumatic sacral pseudomeningocele in the presence of spina bifida occulta. A pseudomeningocele in the sacral region is associated with trauma and with Marfan syndrome, but only one occurrence has been reported in association with spinal dysraphism. Trauma resulted in bleeding into the pseudomeningocele and retrograde passage of blood and fat into the ventricles. An oculomotor nerve palsy subsequently developed in the patient. The authors suspected a subarachnoid hemorrhage caused by a posterior communicating artery aneurysm, although this hypothesis was refuted on further investigation. The pseudomeningocele was drained by direct exposure of the neck and opening of the sac. Postoperatively, communicating hydrocephalus developed and the patient underwent ventriculoperitoneal shunt placement, resulting in resolution of the cranial nerve palsies. This first report of intradural bleeding from direct trauma to a pseudomeningocele illustrates the rare phenomenon of retrograde passage of blood from the sacral region to the brain. It also illustrates a possible but unlikely differential diagnosis of intraventricular blood and fat.

  4. Spina bifida occulta as a relative contraindication for percutaneous retrograde lead insertion for sacral nerve root stimulation.

    Science.gov (United States)

    Michael, L Madison; Whitworth, Louis A; Feler, Claudio A

    2002-01-01

    Percutaneous retrograde lead insertion for sacral nerve root stimulation is a newly described technique being applied to a variety of pain disorders. The success of the procedure rests in a defined epidural space such that there is unimpeded progression of the lead into the desired location. It is hypothesized that any condition that results in anatomic compromise of the epidural space would prevent the success of the procedure. Two patients with biopsy-proven interstitial cystitis and intractable pain were referred to the senior author for evaluation. Percutaneous retrograde lead insertion for sacral nerve root stimulation was performed on these patients in a standard fashion (1,2). Intraoperative fluoroscopy verified the diagnosis of spina bifida occulta. In one patient, implantation was completed percutaneously, but later two of the leads were found to have been placed intradurally. In another patient, repeated attempts at passing the epidural lead distal to the congenital defect were unsuccessful, and the percutaneous procedure was aborted. In conclusion, we have found that the diagnosis of spina bifida occulta, or any other condition in which the epidural space is anatomically disrupted, is a relative contraindication for this procedure. Preoperative roentograms of the lumbar spine may be helpful in avoiding technical difficulties due to this diagnosis.

  5. A Hydatid Cyst of the Lumbar Spine: A Rare Cause of Paraplegia

    Directory of Open Access Journals (Sweden)

    Suhail Ur Rehman

    2017-05-01

    Full Text Available Introduction Hydatid cyst is a zoonotic disease, affecting humans and other mammals worldwide. It is caused by tapeworms of the genus Echinococcus, which is most frequently encountered in the liver and lungs. Although involvement of the central nervous system and spine is rare, it can lead to severe neurological deficits due to direct compression. Case Presentation We report a case of intradural extramedullary hydatid cyst in the lumbar region with a sudden onset, causing progressive paraplegia and areflexia over the past 20 days. After surgical removal, the cyst was sent for histopathological examination. The results showed inner laminated membranes and an outer fibrous layer, surrounded by foreign-body giant cells. The primary objective during surgery was to avoid perforation of the cyst, thereby reducing the risk of systemic dissemination and local seeding of the parasite. During the postoperative period, there was a steady improvement in the neurological deficit, and the patient was discharged with anthelmintics to prevent any distant dissemination. Conclusions An accurate and precise diagnosis is necessary when dealing with cystic pathologies.

  6. Spinal meningeal cyst: analysis with low-field MRI

    International Nuclear Information System (INIS)

    Wu Hongzhou; Chen Yejia; Chen Ronghua; Chen Yanping

    2010-01-01

    Objective: To analyze the characteristics of spinal meningeal cyst in low-field MRI and to discuss its classification, subtype, clinical presentation, and differential diagnosis. Methods: Forty-two patients (20 male, 22 female) were examined with sagittal T 1 -and T 2 -, axial T 2 -weighted MR imaging. Twelve patients were also examined with contrast-enhanced MRI. Results: The cysts were classified using Nakors' classification as type Ia extradural meningeal cysts (4 patients), type Ib sacral meningeal cysts (32), type II extradural meningeal cysts with spinal nerve root fibers (4), and type III spinal intradural meningeal cysts (2). All 42 spinal meningeal cysts had well-defined boundaries with low T 1 and high T 2 signal intensities similar to cerebral spinal fluid. In type Ia, the lesions were often on the dorsum of mid-lower thoracic spinal cord compressing the spinal cord and displacing the extradural fat. In type Ib, the lesions were in the sacral canal with fat plane between the cyst and dural sac. In type II, the lesions contained nerve roots and were lateral to the dural sac. In type III, the lesions were often on the dorsum of spinal cord compressing and displacing the spinal cord anteriorly. Conclusion: Low-field MRI can clearly display the spinal meningeal cyst. Types Ia and Ib spinal meningeal cysts had typical features and can be easily diagnosed. Types II and III should be differentiated from cystic schwannomas and enterogenous cysts, respectively. (authors)

  7. Spinal Cord Injury due to Tumour or Metastasis in Aragón, Northeastern Spain (1991–2008: Incidence, Time Trends, and Neurological Function

    Directory of Open Access Journals (Sweden)

    Maayken Elizabeth Louise van den Berg

    2017-01-01

    Full Text Available Purpose. Understanding the presentation of spinal cord injury (SCI due to tumours considering population distribution and temporal trends is key to managing SCI health services. This study quantified incidence rates, function scores, and trends of SCI due to tumour or metastasis over an 18-year time period in a defined region in Spain. Methods. A retrospective cohort study included in-and outpatients with nontraumatic SCI due to tumour or metastasis admitted to a metropolitan hospital in Spain between 1991 and 2008. Main outcome measures were crude and age- and sex-adjusted incidence rates, tumour location and type, distribution by spinal level, neurological level of injury, and impairment ASIA scores. Results. Primary tumour or metastasis accounted for 32.5% of nontraumatic SCI with an incidence rate of 4.1 per million population. Increasing rates with age and over time were observed. Major pathology groups were intradural-extramedullary masses from which meningiomas and neurinomas accounted for 40%. Lesions were mostly incomplete with predominant ASIA Grade D. Conclusions. Increasing incidence rates of tumour-related SCI over time in the middle-aged and the elderly suggest a growing need for neurooncology health resources in the future.

  8. Cerebral Vasospasm with Ischemia following a Spontaneous Spinal Subarachnoid Hemorrhage

    Directory of Open Access Journals (Sweden)

    Sophia F. Shakur

    2013-01-01

    Full Text Available Cerebral vasospasm is a well-known consequence of aneurysmal subarachnoid hemorrhage (SAH triggered by blood breakdown products. Here, we present the first case of cerebral vasospasm with ischemia following a spontaneous spinal SAH. A 67-year-old woman, who was on Coumadin for atrial fibrillation, presented with chest pain radiating to the back accompanied by headache and leg paresthesias. The international normalized ratio (INR was 4.5. Ten hours after presentation, she developed loss of movement in both legs and lack of sensation below the umbilicus. Spine MRI showed intradural hemorrhage. Her coagulopathy was reversed, and she underwent T2 to T12 laminectomies. A large subarachnoid hematoma was evacuated. Given her complaint of headache preoperatively and the intraoperative finding of spinal SAH, a head CT was done postoperatively that displayed SAH in peripheral sulci. On postoperative day 5, she became obtunded. Brain MRI demonstrated focal restricted diffusion in the left frontoparietal area. Formal angiography revealed vasospasm in anterior cerebral arteries bilaterally and right middle cerebral artery. Vasospasm was treated, and she returned to baseline within 48 hours. Spontaneous spinal SAH can result in the same sequelae typically associated with aneurysmal SAH, and the clinician must have a degree of suspicion in such patients. The pathophysiological mechanisms underlying cerebral vasospasm may explain this unique case.

  9. Post-Operative Intensive Care Unit Requirements Following Elective Craniotomy

    Science.gov (United States)

    HANAK, BRIAN W.; WALCOTT, BRIAN P.; NAHED, BRIAN V.; MUZIKANSKY, ALONA; MIAN, MATTHEW K.; KIMBERLY, WILLIAM T.; CURRY, WILLIAM T.

    2012-01-01

    Objective Commonly, patients undergoing craniotomy are admitted to an intensive care setting post-operatively to allow for close monitoring. We aim to determine the frequency with which patients who have undergone elective craniotomies require intensive care unit level interventions or experience significant complications during the post-operative period to identify a subset of patients for whom an alternative to ICU level care may be appropriate. Methods Following Institutional Review Board approval, a prospective, consecutive cohort of adult patients undergoing elective craniotomy was established at the Massachusetts General Hospital between the dates of April 2010 and March 2011. Inclusion criteria were intradural operations requiring craniotomy performed on adults (18 years of age or greater). Exclusion criteria were cases of an urgent or emergent nature, patients who remained intubated post-operatively, and patients who had a ventriculostomy drain in place at the conclusion of the case. Results 400 patients were analyzed. Univariate analysis revealed that diabetics (p = 0.00047), patients who required intra-operative blood product administration (p = 0.032), older patients (p craniotomy. Properly selected patients may not require post-craniotomy ICU monitoring. Further study of resource utilization is necessary to validate these preliminary findings, particularly in different hospital types. PMID:23182731

  10. Atypical Central Neurocytoma with Recurrent Spinal Dissemination over a Period of 20 Years: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Tareq A. Juratli

    2013-01-01

    Full Text Available We present an unusual case of a late recurrent central neurocytoma that was rediagnosed as an ependymoma and neurocytoma in accordance with changes in histological classifications. Case Description. A 56-year-old male teacher presented with incomplete transverse syndrome due to several intradural extramedullary tumors at the level of lumbar vertebrae 1–3. The histological diagnosis at the time was atypical ependymoma. One year later, two additional tumors were removed at the L5-S1 vertebral level. For 12 years, the patient remained tumor free on followup. Fourteen years after the initial diagnosis, the patient presented with thoracic paresthesias due to two new extramedullary tumors in the C7-T1 and the T8-T9 vertebral levels. After complete removal of the tumors, a radiological survey revealed an intracranial lesion in the third ventricle. Five months later, an additional lesion recurrence was removed surgically. The most recent histological diagnosis revealed an atypical central neurocytoma. In retrospect, the previous tumors were reclassified as neurocytoma according to the additional immunohistochemistry evidence. Discussion. There is no standard adjuvant treatment regimen for atypical neurocytoma; therefore, the patient is currently under close followup. Modern histopathological diagnosis is essential in these cases. Potential routes for dissemination of the tumor should be considered upon first recurrence.

  11. US Pilot Study of Lumbar to Sacral Nerve Rerouting to Restore Voiding and Bowel Function in Spina Bifida: 3-Year Experience

    Directory of Open Access Journals (Sweden)

    Kenneth M. Peters

    2014-01-01

    Full Text Available Objective. To report our experience with creating a skin-central nervous system-bladder reflex arc with intradural lumbar to sacral motor root microanastomosis to restore bladder/bowel function in spina bifida patients. Methods. Urinary/bowel changes from baseline to three years were evaluated with questionnaires, voiding diaries, urodynamics (UDS, and renal function studies. Treatment response was defined as CIC ≤ once/day with stable renal function, voiding efficiency > 50%, and no worsening of motor function. Results. Of 13 subjects (9 female, median age 8 years, 3 voided small amounts at baseline, one voided 200 cc (voiding efficiency 32%, 4/13 reported normal bowels, and 2/13 were continent of stool. Postoperatively, all had transient lower extremity weakness; one developed permanent foot drop. Over three years, renal function remained stable and mean maximum cystometric capacity (MCC increased (P=0.0135. In the 10 that returned at 3 years, 7 were treatment responders and 9 had discontinued antimuscarinics, but most still leaked urine. Only 2/8 with baseline neurogenic detrusor overactivity (NDO still had NDO, all 3 with compliance <10 mL/cm H2O had normalized, 7/10 considered their bowels normal, 5/10 were continent of stool, and 8/10 would undergo the procedure again. Conclusion. Lumbar to sacral nerve rerouting can improve elimination in spina bifida patients. This trial is registered with ClinicalTrials.gov NCT00378664.

  12. Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification

    Energy Technology Data Exchange (ETDEWEB)

    Tortori-Donati, P.; Rossi, A. [Dept. of Paediatric Neuroradiology, G. Gaslini Children' s Research Hospital, Genova (Italy); Cama, A. [Dept. of Paediatric Neurosurgery, G. Gaslini Children' s Research Hospital, Genova (Italy)

    2000-07-01

    Our purpose was to review the neuroradiological features of spinal dysraphism and to correlate them with clinical findings and up-to-date embryological theory. We also aimed to formulate a working classification which might prove useful in clinical practice. We reviewed series of 986 children referred to our Spina Bifida Centre in the past 24 years. There were 353 children with open spinal (OSD) and 633 with closed (skin-covered) spinal (CSD) dysraphism. By far the most common open abnormality was myelomeningocele, and all patients with OSD had a Chiari II malformation. CSD was categorised clinically, depending on the presence of a subcutaneous mass in the back. CSD with a mass mainly consisted of lipomas with dural defects and meningoceles, and accounted for 18.8 % of CSD. CSD without a mass were simple (tight filum terminale, intradural lipoma) or complex (split cord malformations, caudal regression). Our suggested classification is easy to use and to remember and takes into account clinical and MRI features; we have found it useful and reliable when making a preoperative neuroradiological diagnosis in clinical practice. (orig.)

  13. Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification

    International Nuclear Information System (INIS)

    Tortori-Donati, P.; Rossi, A.; Cama, A.

    2000-01-01

    Our purpose was to review the neuroradiological features of spinal dysraphism and to correlate them with clinical findings and up-to-date embryological theory. We also aimed to formulate a working classification which might prove useful in clinical practice. We reviewed series of 986 children referred to our Spina Bifida Centre in the past 24 years. There were 353 children with open spinal (OSD) and 633 with closed (skin-covered) spinal (CSD) dysraphism. By far the most common open abnormality was myelomeningocele, and all patients with OSD had a Chiari II malformation. CSD was categorised clinically, depending on the presence of a subcutaneous mass in the back. CSD with a mass mainly consisted of lipomas with dural defects and meningoceles, and accounted for 18.8 % of CSD. CSD without a mass were simple (tight filum terminale, intradural lipoma) or complex (split cord malformations, caudal regression). Our suggested classification is easy to use and to remember and takes into account clinical and MRI features; we have found it useful and reliable when making a preoperative neuroradiological diagnosis in clinical practice. (orig.)

  14. Lumbar spinal imaging in radicular pain and related conditions. Understanding diagnostic images in a clinical context

    International Nuclear Information System (INIS)

    Wilmink, Jan T.

    2010-01-01

    There is general agreement that lumbosacral nerve root compression is a prime factor in the pathogenesis of sciatica and neurogenic claudication, although humoral and vascular factors certainly play a role as well. This book focuses on imaging of the various ways in which nerve root compression can come about, and assessing which anatomic features are reliably associated with the occurrence of radicular pain, as opposed to morphologic findings which are probably coincidental. After a discussion of the nature of radicular pain and related symptoms, spinal imaging techniques and options are reviewed, with emphasis on the role of MR myelography in assessing the condition of the intradural nerve roots. A chapter on normal topographic, sectional, and functional (dynamic) radiologic anatomy is followed by a presentation on pathologic anatomy, addressing the various mechanisms of nerve root compression. In the chapter on pre- and postoperative imaging, features which may help to predict the evolution of the symptoms are discussed, with an eye to selecting candidates for surgical treatment. This is followed by a discussion of the role and limitations of imaging studies in various adverse postoperative conditions. In illustrations involving patient studies, imaging features are linked where possible to the clinical symptoms and history of the individuals involved. (orig.)

  15. Lumbar spinal imaging in radicular pain and related conditions. Understanding diagnostic images in a clinical context

    Energy Technology Data Exchange (ETDEWEB)

    Wilmink, Jan T. [University Hospital Maastricht (Netherlands). Dept. Radiology

    2010-07-01

    There is general agreement that lumbosacral nerve root compression is a prime factor in the pathogenesis of sciatica and neurogenic claudication, although humoral and vascular factors certainly play a role as well. This book focuses on imaging of the various ways in which nerve root compression can come about, and assessing which anatomic features are reliably associated with the occurrence of radicular pain, as opposed to morphologic findings which are probably coincidental. After a discussion of the nature of radicular pain and related symptoms, spinal imaging techniques and options are reviewed, with emphasis on the role of MR myelography in assessing the condition of the intradural nerve roots. A chapter on normal topographic, sectional, and functional (dynamic) radiologic anatomy is followed by a presentation on pathologic anatomy, addressing the various mechanisms of nerve root compression. In the chapter on pre- and postoperative imaging, features which may help to predict the evolution of the symptoms are discussed, with an eye to selecting candidates for surgical treatment. This is followed by a discussion of the role and limitations of imaging studies in various adverse postoperative conditions. In illustrations involving patient studies, imaging features are linked where possible to the clinical symptoms and history of the individuals involved. (orig.)

  16. Spinal tumours in neurofibromatosis type 1: an MRI study of frequency, multiplicity and variety

    International Nuclear Information System (INIS)

    Thakkar, S.D.; Mautner, V.F.; Feigen, U.

    1999-01-01

    In neurofibromatosis type 1 (NF1) spinal tumours cause neurological symptoms in about 2 % of patients. Among over 1400 patients with NF1 we saw symptomatic spinal tumours in 23 (1.6 %). MRI of the entire spinal canal was obtained in 54 patients aged 5-56 years with NF1. The number, site, morphology and signal characteristics of the spinal tumours were recorded and analysed. There were 24 patients with symptoms such as sensory impairment or paralysis; 30 patients had no neurological deficits. Of the 24 symptomatic patients, 23 (96 %) had spinal tumours, while we saw spinal tumours in 12 (40 %) of the 30 patients without neurological deficits. No spinal segment was preferred in symptomatic or asymptomatic patients. Most intraspinal extramedullary tumours were primarily extradural and intraforaminal. MRI showed intramedullary tumours in 3 patients (6 %), intraspinal extramedullary tumours in 18 (33 %) and intraforaminal tumours in 31 (57 %). Only neurological deficits in patients with NF1 should prompt further diagnostic clarification. In patients with neurological symptoms there may be a multiplicity of masses in the spinal canal, which can lead to difficulties in attaching symptoms to a certain tumour. In patients who do not satisfy the NIH criteria, it can be a helpful observation that spinal tumours in NF1 are primarily intraforaminal, extending into the spinal canal, while in NF2 they are mostly intraspinal intradural tumours. (orig.)

  17. A meningioma and its consequences for American history and the rise of neurosurgery.

    Science.gov (United States)

    Ansari, Shaheryar F; Gianaris, Nicholas G; Cohen-Gadol, Aaron A

    2011-12-01

    The case of General Leonard Wood is notable both for its contribution to the field of neurosurgery and its historical significance. As one of Harvey Cushing's first successful brain tumor operations in 1910, Wood's surgery was part of the case series that culminated in Cushing's monograph Meningioma. This case was important to the rise of Cushing's career and his recognition as a member of the next generation of neurosurgeons who did not settle for mere bony decompression to taper intracranial tension but who dared to pursue intradural resections-operations that had been performed by surgeons for decades but were frowned upon because of the attendant risks. Cushing's operation to remove a recurrent brain tumor ended Wood's life in 1927. The authors discuss the effects the tumor may have had on Wood's life and career, explore an alternate explanation for the cause of Wood's death, and provide a brief account of the life of General Wood, highlighting events in his military and administrative career juxtaposed against the progression of his illness. Furthermore, the case history of the General is reviewed, using information drawn from the original patient notes and recently discovered images from the Cushing Brain Tumor Registry that elucidate more details about General Wood's story, from the injury that caused his first tumor to his final surgery, leading to his demise.

  18. An Unusual Case of Systemic Inflammatory Myofibroblastic Tumor with Successful Treatment with ALK-Inhibitor

    Directory of Open Access Journals (Sweden)

    Sanjivini V. Jacob

    2014-01-01

    Full Text Available Systemic inflammatory myofibroblastic tumor is an exceedingly rare entity. A 45-year-old Hispanic female presented with a 6-month history of left-sided thigh pain, low back pain, and generalized weakness. PET/CT scan revealed abnormal activity in the liver, adrenal gland, and pancreas. MRI of the abdomen demonstrated two 6-7 cm masses in the liver. MRI of the lumbar spine demonstrated lesions in the L2 to L4 spinous processes, paraspinal muscles, and subcutaneous tissues, as well as an 8 mm enhancing intradural lesion at T11, all thought to be metastatic disease. A biopsy of the liver showed portal tract expansion by a spindle cell proliferation rich in inflammation. Tumor cells showed immunoreactivity for smooth muscle actin and anaplastic lymphoma kinase 1 (ALK1. Tissue from the L5 vertebra showed a process histologically identical to that seen in the liver. FISH analysis of these lesions demonstrated an ALK (2p23 gene rearrangement. The patient was successfully treated with an ALK-inhibitor, Crizotinib, and is now in complete remission. We present the first reported case, to our knowledge, of inflammatory myofibroblastic tumor with systemic manifestations and ALK translocation. This case is a prime example of how personalized medicine has vastly improved patient care through the use of molecular-targeted therapy.

  19. Clinical potential and limitation of MRI for degenerative lumbar spinal diseases. Comparison of MRI, myelography, CT and selective nerve root infiltration

    Energy Technology Data Exchange (ETDEWEB)

    Seki, Michihiro; Kikuchi, Shinichi [Fukushima Medical Coll. (Japan)

    1994-12-01

    To assess the clinical potential and limitations of magnetic resonance imaging (MRI) in degenerative lumbar spinal diseases, the findings of MR imaging were compared with those of myelography and CT. The subjects were 80 patients with intervertebral disc herniation (46), spondylosis (28), degenerative spondylolisthesis (5), and spondylolysis (one). There was a good correlation between sagittal MRI (T1-weighted images) and myelography in measuring the anteroposterior diameter and the compression rate of the injured dural canal in all disease categories. However, MRI was inferior, irrespective of sagittal and coronal images, to myelography in detecting blocking of the dural canal and intradural findings such as redundant nerve roots. MRI was inferior to selective nerve root infiltration in visualizing the compression of the nerve root, irrespective of diseases; however, there was no difference in abnormal findings of the running of nerve root between the two modalities. Transverse MRI was superior to CT in visualizing the nerve root. Thus, MRI alone is insufficient for the diagnosis of degenerative lumbar spinal diseases, and the other modalities should be supplementary for pathophysiological understanding of these diseases. (N.K.).

  20. Clinical potential and limitation of MRI for degenerative lumbar spinal diseases. Comparison of MRI, myelography, CT and selective nerve root infiltration

    International Nuclear Information System (INIS)

    Seki, Michihiro; Kikuchi, Shinichi

    1994-01-01

    To assess the clinical potential and limitations of magnetic resonance imaging (MRI) in degenerative lumbar spinal diseases, the findings of MR imaging were compared with those of myelography and CT. The subjects were 80 patients with intervertebral disc herniation (46), spondylosis (28), degenerative spondylolisthesis (5), and spondylolysis (one). There was a good correlation between sagittal MRI (T1-weighted images) and myelography in measuring the anteroposterior diameter and the compression rate of the injured dural canal in all disease categories. However, MRI was inferior, irrespective of sagittal and coronal images, to myelography in detecting blocking of the dural canal and intradural findings such as redundant nerve roots. MRI was inferior to selective nerve root infiltration in visualizing the compression of the nerve root, irrespective of diseases; however, there was no difference in abnormal findings of the running of nerve root between the two modalities. Transverse MRI was superior to CT in visualizing the nerve root. Thus, MRI alone is insufficient for the diagnosis of degenerative lumbar spinal diseases, and the other modalities should be supplementary for pathophysiological understanding of these diseases. (N.K.)

  1. Unruptured aneurysms with cranial nerve symptoms: efficacy of endosaccular guglielmi detachable coil treatment

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dong Joon; Kim, Dong Ik; Lee, Seung Koo; Kim, Si Yeon [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2003-09-01

    To evaluate the efficacy of endosaccular Guglielmi detachable coil (GDC) treatment of unruptured aneurysms causing cranial nerve (CN) symptoms. Among a database of 218 patients whose aneurysms were treated using GDC, seven patients met the criteria for unruptured aneurysms presenting with symptoms and signs of CN palsy. Changes in CN symptoms before and after GDC treatment were reviewed. Aneurysms were located in the internal carotid-posterior communicating artery (n=3), the basilar bifurcation (n=1) and the cavernous internal carotid artery (n=3). CN symptoms included ptosis (n=6), mydriasis (n=2), and extraocular muscle (EOM) disorder (CN III: n=4; CN VI: n=3). Overall, improvement or resolution of CN symptoms after treatment was noted in five patients. CN symptoms in cases involving small (10 mm) and intradural aneurysms tended to respond better to GDC treatment. Ptosis was the initial symptom to show improvement, while EOM dysfunction responded least favourably. GDC coil packing appears to be an appropriate treatment method for the relief of CN symptoms associated with intracranial aneurysms.

  2. A road map to the internal carotid artery in expanded endoscopic endonasal approaches to the ventral cranial base.

    Science.gov (United States)

    Labib, Mohamed A; Prevedello, Daniel M; Carrau, Ricardo; Kerr, Edward E; Naudy, Cristian; Abou Al-Shaar, Hussam; Corsten, Martin; Kassam, Amin

    2014-09-01

    Injuring the internal carotid artery (ICA) is a feared complication of endoscopic endonasal approaches. To introduce a comprehensive ICA classification scheme pertinent to safe endoscopic endonasal cranial base surgery. Anatomic dissections were performed in 33 cadaveric specimens (bilateral). Anatomic correlations were analyzed. Based on anatomic correlations, the ICA may be described as 6 distinct segments: (1) parapharyngeal (common carotid bifurcation to ICA foramen); (2) petrous (carotid canal to posterolateral aspect of foramen lacerum); (3) paraclival (posterolateral foramen lacerum to the superomedial aspect of the petrous apex); (4) parasellar (superomedial petrous apex to the proximal dural ring); (5) paraclinoid (from the proximal to the distal dural rings); and (6) intradural (distal ring to ICA bifurcation). Corresponding surgical landmarks included the Eustachian tube, the fossa of Rosenmüller, and levator veli palatini for the parapharyngeal segment; the vidian canal and V3 for the petrous segment; the fibrocartilage of foramen lacerum, foramen rotundum, maxillary strut, lingular process of the sphenoid bone, and paraclival protuberance for the paraclival segment; the sellar floor and petrous apex for the parasellar segment; and the medial and lateral opticocarotid and lateral tubercular recesses, as well as the distal osseous arch of the carotid sulcus for the paraclinoid segment. The proposed endoscopic classification outlines key anatomic reference points independent of the vessel's geometry or the sinonasal pneumatization, thus serving as (1) a practical guide to navigate the ventral cranial base while avoiding injury to the ICA and (2) further foundation for a modular access system.

  3. Holmium laser enucleation of the prostate and retropubic prostatic adenomectomy: morbidity analysis and anesthesia considerations.

    Science.gov (United States)

    Soto-Mesa, D; Amorín-Díaz, M; Pérez-Arviza, L; Fernández-Pello Montes, S; Martín-Huéscar, A

    2015-11-01

    Holmium laser enucleation of the prostate (HoLEP) is an alternative to prostatic adenomectomy for the surgical treatment of benign prostatic hypertrophy. We analyzed our learning curve for this technique, and we compared it in a secondary manner with prostatic adenomectomy. A retrospective comparative study was conducted that included the first 100 cases of HoLEP performed in our center and the latest 50 cases of retropubic adenomectomy. We collected data on the patients, the surgery, the anesthesia, the perioperative variables, the anesthesia complications and the postoperative variables, with a 6-month follow-up. We analyzed the learning curve without mentors for HoLEP and compared the characteristics of HoLEP in 2 separate phases (learning and stabilization phases) with the latest retropubic prostatic adenomectomies performed. Intradural anesthesia was the most common technique. The transfusion needs, length of stay (PLocal anesthesia is a good choice for the anesthesia technique. Copyright © 2014 AEU. Publicado por Elsevier España, S.L.U. All rights reserved.

  4. Primary reconstruction of pelvic floor defects following sacrectomy using Permacol graft.

    Science.gov (United States)

    Abhinav, K; Shaaban, M; Raymond, T; Oke, T; Gullan, R; Montgomery, A C V

    2009-04-01

    The large pelvic floor defect following sacrectomy for sacral masses leaves the challenging problem of primary closure and herniation. We present the outcome of primary repair using Permacol, a biomaterial made of acellular porcine cross-linked dermal collagen and with similar tensile strength to polypropylene mesh. It is non-allergenic and possibly less likely than synthetic mesh to cause inflammation leading to small bowel adherence; fistula formation and graft extrusion. Following implantation, Permacol is colonized by host cells and resists degradation by host enzymes. Three patients had sacrectomy with primary repair of pelvic floor defects between March 2004 and August 2005. Two had excision of sacral chordomas and one excision of a sacrococcygeal teratoma. Repair of the defect was carried out using the Permacol graft, suturing to the sacrum, anococcygeal raphe and ischial spines. Two suction drains were placed superficial to the mesh. All patients had gross en-bloc tumour resections and over a median follow-up period of 1year (range 8-25months), there were no complications related to primary repair. Primary closure of a large defect following sacrectomy using a Permacol graft, in our early experience seems to be convenient and safe without the development of herniation.

  5. Diagnosis and treatment of isolated sphenoid sinus disease: a review of 109 cases.

    Science.gov (United States)

    Socher, Jan Alessandro; Cassano, Michele; Filheiro, Claudeomiro Augustinho; Cassano, Pasquale; Felippu, Alexandre

    2008-09-01

    Endoscopic transethmoidal sphenoidotomy performed mainly in the early stages of the pathology and by expert hands is [WX1]very effective in treating isolated sphenoid sinus disease. This study aimed to investigate the causes of isolated sphenoid sinus disease identified in 109 patients and report on the most appropriate diagnostic and therapeutic patterns for an earlier diagnosis and a successful treatment of the disease. A total of 109 subjects with various isolated sphenoid pathologies were first examined by general objective examination, nasal sinus endoscopy, CT scan of paranasal sinuses, and in some cases with MRI. Then, they underwent medical and/or surgical treatment. Nineteen patients (17.43%) had isolated sphenoiditis, 6 (5.5%) fungal sinusitis, 30 (27.52%) mucocele, 6 (5.5%) fibrous dysplasia, 6 (5.5%) meningoencephalocele, 5 (4.58%) inverted papilloma, 4 (3.66%) epidermoid carcinoma, 10 (9.17%) liquor fistula, 1 (0.9%) rhabdomyosarcoma, 1 (0.9%) chordoma, and 1 (0.9%) had carotid pseudoaneurysm. Evidence of definitive diagnosis by endoscopy was obtained in less than half of the cases. CT scan, however, sometimes in combination with MRI, determined the pathology in all the cases. A follow-up of at least 4 years post-surgery showed good results in all the patients who underwent endoscopic transethmoidal sphenoidotomy.

  6. Unilateral duplicated abducens nerve coursing through both the sphenopetroclival venous gulf and cavernous sinus: a case report.

    Science.gov (United States)

    Coquet, Thomas; Lefranc, Michel; Chenin, Louis; Foulon, Pascal; Havet, Éric; Peltier, Johann

    2018-03-15

    In this anatomy report, we describe the first case of abducens nerve duplication limited to the sphenopetroclival venous gulf and the cavernous sinus. The objective point of division of the two duplicated roots was localized at the gulfar face of the dural porus, just distal to the unique cisternal trunk of the abducens nerve, as it pierced the petroclival dural mater. In the gulfar segment, both roots traveled through a variant of Dorello's canal called the "petrosphenoidal canal" and remained separated through the posterior half of the cavernous sinus. Both roots finally fused in the anterior half of the cavernous sinus to innervate the lateral rectus muscle as a single trunk. Although many variants of the abducens nerve have been reported over the recent decades, this anatomic variation has never been previously described and enriches the continuum of abducens nerve variations reported in the literature data. Awareness of this variation is crucial for neurosurgeons, especially during clival or petrosal surgical approaches used for resection of skull base chordomas.

  7. Percutaneous gastrostomy -a report of twenty-seven cases-

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Tae Ho; Lee, Ho Suk; Kim, Yong Joo; Kim, Tae Hun; Suh, Kyung Jin; Kang, Duk Sik [College of Medicine, Kyungpook National University, Daegu (Korea, Republic of)

    1991-05-15

    Nutritional support by gastrostomy feeding is an important treatment adjunct when major swallowing difficulty or debilitating diseases is present. This technique for percutaneous placement of a gastric feeding tube now provides many patients with a simple, safe, and well-tolerated alternative to surgical feeding gastrostomy. We experienced 27 cases of percutaneous gastrostomy from January 1989 to February 1991 at Kyungpook National University Hospital. The underlying diseases of the patients were esophageal cancer (11), pyriform sinus cancer (4), laryngeal cancer (3), tongue cancer (2), lye stricture (1), lung cancer (1), chordoma (1), lethal midline granuloma (1), malignant lymphoma (1), maxillary cancer (1), and tonsil cancer (1). Selding method was used in all cases. In five patients it was difficult to insert the nasogastric tube for air insufflation, and one patient had subtotal gastrectomy with gastrojejunostomy, but gastrostomy was performed in all cases. The general condition improved in all patients. We experienced one case of mild peritonitis, but no other significant complications were observed. Gastrostomy tubes were patient in all patients during their survival period, except two patients who experienced obstruction 2 and 6 months after the procedure. Percutaneous gastrostomy can be used safely and effectively in such patients who have pharyngoesophageal or CNS lesions precluding oral intake.

  8. Acute radiation-induced toxicity of heavy ion radiotherapy delivered with intensity modulated pencil beam scanning in patients with base of skull tumors.

    Science.gov (United States)

    Schulz-Ertner, Daniela; Haberer, Thomas; Scholz, Michael; Thilmann, Christoph; Wenz, Frederik; Jäkel, Oliver; Kraft, Gerhard; Wannenmacher, Michael; Debus, Jürgen

    2002-08-01

    The purpose of this paper is to evaluate acute radiation-induced toxicity of carbon ion therapy. From December 1997 to November 2000, 37 patients with chordomas and low-grade chondrosarcomas of the skull base have been treated with carbon ions at the heavy ion synchrotron (SIS) at GSI, Darmstadt. Tumor-conformal application of carbon beams was realized by intensity-controlled raster scanning in combination with pulse-to-pulse energy variation. The treatment planning procedure included a biological plan optimization. We applied a cobalt-Gray equivalent dose of 60GyE. Acute toxicity was assessed according to the common toxicity criteria (CTC). Acute toxicity included skin reactions ( degrees I+ degrees II) in four patients, mucositis ( degrees I- degrees III) in eight patients, otitis and middle ear effusion in four, sinusitis in four, nausea/weight loss in one and edema of the temporal lobes in one patient. In two patients, preexisting neurological symptoms worsened. We did not observe acute morbidity > degrees III of normal tissues. Scanning beam delivery of heavy charged particles is safe and reliable. No unexpected acute dose limiting toxicity was observed. With regard to toxicity, a substantial improvement compared to passive beam shaping technology is achieved.

  9. Tumor Cells Surviving Exposure to Proton or Photon Radiation Share a Common Immunogenic Modulation Signature, Rendering Them More Sensitive to T Cell–Mediated Killing

    Energy Technology Data Exchange (ETDEWEB)

    Gameiro, Sofia R.; Malamas, Anthony S. [Laboratory of Tumor Immunology and Biology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland (United States); Bernstein, Michael B. [Division of Radiation Oncology, M. D. Anderson Cancer Center, Houston, Texas (United States); Tsang, Kwong Y. [Laboratory of Tumor Immunology and Biology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland (United States); Vassantachart, April; Sahoo, Narayan; Tailor, Ramesh; Pidikiti, Rajesh [Division of Radiation Oncology, M. D. Anderson Cancer Center, Houston, Texas (United States); Guha, Chandan P. [Department of Radiation Oncology, Montefiore Medical Center, Bronx, New York (United States); Hahn, Stephen M.; Krishnan, Sunil [Division of Radiation Oncology, M. D. Anderson Cancer Center, Houston, Texas (United States); Hodge, James W., E-mail: jh241d@nih.gov [Laboratory of Tumor Immunology and Biology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland (United States)

    2016-05-01

    Purpose: To provide the foundation for combining immunotherapy to induce tumor antigen–specific T cells with proton radiation therapy to exploit the activity of those T cells. Methods and Materials: Using cell lines of tumors frequently treated with proton radiation, such as prostate, breast, lung, and chordoma, we examined the effect of proton radiation on the viability and induction of immunogenic modulation in tumor cells by flow cytometric and immunofluorescent analysis of surface phenotype and the functional immune consequences. Results: These studies show for the first time that (1) proton and photon radiation induced comparable up-regulation of surface molecules involved in immune recognition (histocompatibility leukocyte antigen, intercellular adhesion molecule 1, and the tumor-associated antigens carcinoembryonic antigen and mucin 1); (2) proton radiation mediated calreticulin cell-surface expression, increasing sensitivity to cytotoxic T-lymphocyte killing of tumor cells; and (3) cancer stem cells, which are resistant to the direct cytolytic activity of proton radiation, nonetheless up-regulated calreticulin after radiation in a manner similar to non-cancer stem cells. Conclusions: These findings offer a rationale for the use of proton radiation in combination with immunotherapy, including for patients who have failed radiation therapy alone or have limited treatment options.

  10. Treatment of pediatric patients and young adults with particle therapy at the Heidelberg Ion Therapy Center (HIT): establishment of workflow and initial clinical data

    International Nuclear Information System (INIS)

    Combs, Stephanie E; Witt, Olaf; Jäkel, Oliver; Kulozik, Andreas E; Debus, Jürgen; Kessel, Kerstin A; Herfarth, Klaus; Jensen, Alexandra; Oertel, Susanne; Blattmann, Claudia; Ecker, Swantje; Hoess, Angelika; Martin, Eike

    2012-01-01

    To report on establishment of workflow and clinical results of particle therapy at the Heidelberg Ion Therapy Center. We treated 36 pediatric patients (aged 21 or younger) with particle therapy at HIT. Median age was 12 years (range 2-21 years), five patients (14%) were younger than 5 years of age. Indications included pilocytic astrocytoma, parameningeal and orbital rhabdomyosarcoma, skull base and cervical chordoma, osteosarcoma and adenoid-cystic carcinoma (ACC), as well as one patient with an angiofibroma of the nasopharynx. For the treatment of small children, an anesthesia unit at HIT was established in cooperation with the Department of Anesthesiology. Treatment concepts depended on tumor type, staging, age of the patient, as well as availability of specific study protocols. In all patients, particle radiotherapy was well tolerated and no interruptions due to toxicity had to be undertaken. During follow-up, only mild toxicites were observed. Only one patient died of tumor progression: Carbon ion radiotherapy was performed as an individual treatment approach in a child with a skull base recurrence of the previously irradiated rhabdomyosarcoma. Besides this patient, tumor recurrence was observed in two additional patients. Clinical protocols have been generated to evaluate the real potential of particle therapy, also with respect to carbon ions in distinct pediatric patient populations. The strong cooperation between the pediatric department and the department of radiation oncology enable an interdisciplinary treatment and stream-lined workflow and acceptance of the treatment for the patients and their parents

  11. REVIEW OF NASA FIBROMYXOMA: Aggressive Behaviour? (Case Report

    Directory of Open Access Journals (Sweden)

    Rr. Suzy Indharti

    2015-12-01

    Full Text Available Background: Myxomas are rare benign tumors arising from mesenchymal tissues throughout the body. These tumors are usually seen in the atrium of the heart and the jawbone. Involvement of the skull base with intracranial extension is extremely rare, and only a few cases of primary intracranial myxomas have been described in the literature. This article presents a rare case of primary myxoma of the nasal bone. Methods: The patient underwent a skull base surgery with a pre-diagnosis of possible fibromyxomas. The tumor pathology revealed a diagnosis of myxoma with bone and meningeal involvement. Despite the debulking surgery, the tumor showed a local recurrence in five month. A second debulking surgery in piece meal was required. In the article, the etiology, histological and radiological findings as well as treatment options of this rare entity were briefly discussed under the highlights of the relevant literature. Such a localization and intracranial extension of myxomas is extremely unusual in clinical practice; the diagnosis therefore requires a high degree of suspicion and detailed histopathological examination. The differential diagnosis frequently includes chondrosarcomas, chordoma, metastatic tumors of the skull, hemangiopericytoma, meningioma and other neoplasms of the dura and skull base in this location.

  12. Imaging of cerebellopontine angle lesions: an update. Part 2: intra-axial lesions, skull base lesions that may invade the CPA region, and non-enhancing extra-axial lesions

    Energy Technology Data Exchange (ETDEWEB)

    Bonneville, Fabrice; Chiras, Jacques [Pitie-Salpetriere Hospital, Department of Neuroradiology, Paris (France); Savatovsky, Julien [Adolphe de Rothschild Foundation, Department of Radiology, Paris (France)

    2007-11-15

    Computed tomography (CT) and magnetic resonance (MR) imaging reliably demonstrate typical features of vestibular schwannomas or meningiomas in the vast majority of mass lesions responsible for cerebellopontine angle (CPA) syndrome. However, a large variety of unusual lesions can also be encountered in the CPA. Covering the entire spectrum of lesions potentially found in the CPA, these articles explain the pertinent neuroimaging features that radiologists need to know to make clinically relevant diagnoses in these cases, including data from diffusion- and perfusion-weighted imaging or MR spectroscopy, when available. A diagnostic algorithm based on the lesion's site of origin, shape and margins, density, signal intensity and contrast material uptake is also proposed. Non-enhancing extra-axial CPA masses are cystic (epidermoid cyst, arachnoid cyst, neurenteric cyst) or contain fat (dermoid cyst, lipoma). Tumours can also extend into the CPA by extension from the skull base (paraganglioma, chondromatous tumours, chordoma, cholesterol granuloma, endolymphatic sac tumour). Finally, brain stem or ventricular tumours can present with a significant exophytic component in the CPA that may be difficult to differentiate from an extra-axial lesion (lymphoma, hemangioblastoma, choroid plexus papilloma, ependymoma, glioma, medulloblastoma, dysembryoplastic neuroepithelial tumour). (orig.)

  13. Extent of Endoscopic Resection for Anterior Skull Base Tumors: An MRI-Based Volumetric Analysis.

    Science.gov (United States)

    Koszewski, Ian J; Avey, Gregory; Ahmed, Azam; Leonhard, Lucas; Hoffman, Matthew R; McCulloch, Timothy M

    2017-06-01

    Objective  To determine the volume of ventral skull base tumor removed following endoscopic endonasal (EEA) resection using MRI-based volumetric analysis and to evaluate the inter-rater reliability of such analysis. Design  Retrospective case series. Setting  Academic tertiary care hospital. Participants  EEA patients November 2012 to August 2015. Main Outcome Measures  Volumetric analysis of pre- and immediately postoperative MR imaging was performed independently by two investigators. The percentage of total tumor resected was evaluated according to resection goal and tumor type. Results  A total of 39 patients underwent resection. Intraclass correlation coefficients between the raters were 0.9988 for preoperative and 0.9819 for postoperative images. Tumors (and average percentage removed) included 17 nonsecreting pituitary adenomas (95.3%), 8 secreting pituitary adenomas (86.2%), 4 meningiomas (81.6%), 3 olfactory neuroblastomas (100%), 2 craniopharyngiomas (100%), 1 large B-cell lymphoma (90.5%), 1 germ cell neoplasm (48.3), 1 benign fibrous connective tissue mass (93.4%), 1 epidermoid cyst (68.4%), and 1 chordoma (100%). For tumors treated with intent for gross total resection, 96.9 ± 4.8% was removed. Conclusion  EEAs achieved tumor resection rates of ∼97% when total resection was attempted. The radiographic finding of residual tumor is of uncertain clinical significance. The volumetric analysis employed in this study demonstrated high inter-rater reliability and could facilitate further study.

  14. Crowned dens syndrome

    Directory of Open Access Journals (Sweden)

    Norma Belfiore

    2012-10-01

    Full Text Available Introduction Microcrystalline deposition in peri-odontoid articular structures is mainly responsible for acute or chronic cervical pain and is known as “crowned dens syndrome”.Materials and methods We described two cases of acute cervical pain associated with onset of fever and peripheral acute monoarthritis. Cervical computed tomography (CT scan showed linear calcification of the retrodens ligament and calcium pyrophosphate dehydrate (CPPD crystals were found in synovial fluid of inflamed joints. Both patients were treated with anti-inflammatory drugs and colchicine.Discussion Calcium depositions around the odontoid process of the axis can be occasionally detected by radiological studies. They are frequently asymptomatic but sometimes can be associated with severe neurological abnormalities, fever and acute neck pain. CPPD crystals are usually deposited in joints and bursae but occasionally can disrupt these anatomical confines and deposit in periarticular tissues, sometimes forming large masses confused with tumours.Conclusions Acute onset of cervical neck pain associated with elevation of inflammatory indicators and/or signs of cervical myelopathy should suggest CT scans searching for microcrystal depositions in the peryodonthoid tissue. Differential diagnosis of fever of unknown origin (FUO should include crowned dens syndrome specially in the elderly after exclusion of several endocrine or metabolic disorders, infection diseases (meningitis, arthritis (psoriatic arthritis and ankylosing spondylitis and tumours (chordoma, meningioma, osteoblastoma.

  15. CT diagnosis of sacral bone tumors

    International Nuclear Information System (INIS)

    Ni Zhaomin; Jin Zhonggao; Zhu Yaoming; Wu Xiao

    2008-01-01

    Objective: To evaluate the CT characteristics of sacral bone tumors. Methods: The CT characteristics of 28 cases with sacral bone tumors were retrospectively analyzed, including 13 cases metastasizes, 4 cases chordomas, 1 case chondrosarcoma, 1 case primitive neurotodemal tumors (PNET), 3 cases osteoblastomas, 1 case osteosarcoma, 2 cases neurilemmomas, 3 cases cysts (1 case of simple bone cyst and 2 cases of intracranial arachnoid cysts). Results: The CT characteristics of sacral bone tumors were as followings: different ranges and location of tumor, sacral bone destruction (lytic destruction for most malignant tumors and expansive for benign tumors), the remains of bone, soft tissue mass, calcification in tumor, sacral canal and sacral foramen obstruction. Benign tumors were often with sharp margin and sclerotic borders (except osteoblastomas), and the malignant tumors were often without clear verge and sclerotic borders. Except the cystic diseases, all the others were enhanced variously after contrast enhanced scanning. Conclusion: CT imaging can clearly display the location, ranges of tumor and the relation between tumor and surrounding tissues. Most sacral bone tumors can be correctly diagnosed in pre-operation according to their different CT characteristics. (authors)

  16. Plasmacytoma of the Clivus Presenting as Bilateral Sixth Nerve Palsy.

    Science.gov (United States)

    Kalwani, Neil; Remenschneider, Aaron K; Faquin, William; Ferry, Judith; Holbrook, Eric H

    2015-07-01

    Background and Importance Plasmacytomas are monoclonal proliferations of plasma cells that may arise within soft tissue or bone. The skull base is a rare site for plasmacytomas to occur, and few cases have been reported in the literature. When present in the skull base, plasmacytomas may result in cranial neuropathies and often progress to multiple myeloma more rapidly than other intracranial or skeletal plasmacytomas. Clinical Presentation A 69-year-old man presented with a primary complaint of diplopia and an examination consistent with bilateral abducens nerve palsy. No other deficits were noted. Magnetic resonance imaging of the skull base demonstrated a large T1 isointense moderately enhancing lesion centered within the clivus. Endoscopic biopsy of the mass revealed sheets and aggregates of mature monoclonal plasma cells. The patient's initial systemic work-up revealed that this was a solitary lesion, and he was treated with radiation therapy to the skull base with a durable local effect at 18-month follow-up. Unfortunately he progressed to multiple myeloma with peripheral osteolytic lesions but has been stabilized on chemotherapeutics. Conclusion The clivus is an unusual site for intracranial plasmacytomas, and enhancing lesions must be differentiated from chordoma. Characteristic findings on histopathology include an immunoglobulin light-chain restricted clonal proliferation of plasma cells. Treatment is most commonly radiotherapy with surgery reserved for biopsy and palliation. Clinicians should be aware of the increased risk of progression to multiple myeloma in skull base plasmacytomas.

  17. The utility of 3D printing for surgical planning and patient-specific implant design for complex spinal pathologies: case report.

    Science.gov (United States)

    Mobbs, Ralph J; Coughlan, Marc; Thompson, Robert; Sutterlin, Chester E; Phan, Kevin

    2017-04-01

    OBJECTIVE There has been a recent renewed interest in the use and potential applications of 3D printing in the assistance of surgical planning and the development of personalized prostheses. There have been few reports on the use of 3D printing for implants designed to be used in complex spinal surgery. METHODS The authors report 2 cases in which 3D printing was used for surgical planning as a preoperative mold, and for a custom-designed titanium prosthesis: one patient with a C-1/C-2 chordoma who underwent tumor resection and vertebral reconstruction, and another patient with a custom-designed titanium anterior fusion cage for an unusual congenital spinal deformity. RESULTS In both presented cases, the custom-designed and custom-built implants were easily slotted into position, which facilitated the surgery and shortened the procedure time, avoiding further complex reconstruction such as harvesting rib or fibular grafts and fashioning these grafts intraoperatively to fit the defect. Radiological follow-up for both cases demonstrated successful fusion at 9 and 12 months, respectively. CONCLUSIONS These cases demonstrate the feasibility of the use of 3D modeling and printing to develop personalized prostheses and can ease the difficulty of complex spinal surgery. Possible future directions of research include the combination of 3D-printed implants and biologics, as well as the development of bioceramic composites and custom implants for load-bearing purposes.

  18. Gluteus Maximus Turnover Flap for Sacral Osteomyelitis After Radiation Therapy.

    Science.gov (United States)

    Ishiwata, Sho; Yanagawa, Takashi; Saito, Kenichi; Takagishi, Kenji

    2015-07-01

    Developments in radiation therapy modalities offer alternative treatments for unresectable malignant tumors in the pelvis and trunk. However, poor vascularity as a result of radiation therapy makes the treated lesion susceptible to infection, and there are no established treatments for pelvic osteomyelitis with a large dead space after radiation therapy. The authors report 2 cases of sacral osteomyelitis after radiation therapy that were treated successfully with a gluteus maximus turnover flap. To create the flap, the distal portion of the lower third of the muscle was detached from the trochanter. The distal edge of the flap was turned toward the sacral defect and sewn to the remnant of the sacrum, which filled the dead space with the muscle bulk. A 68-year-old man with a recurrent sacral chordoma was treated with carbon ion radiation therapy; however, a sacral infection developed 5 months later. Debridement and a course of antibiotics could not control the infection and did not induce sufficient formation of granulation tissue in the large and deep dead space. The turnover flap with both gluteus maximus muscles cured the deep-seated infection and closed the wound. A 58-year-old woman had sacral osteoradionecrosis with infection. A turnover flap created with the left gluteus maximus muscle controlled the infection and closed the wound after the first operation, a V-Y flap, failed. This study showed that a gluteus maximus muscle turnover flap effectively controlled infectious lesions with large and deep dead space around the sacrum. Copyright 2015, SLACK Incorporated.

  19. Tumores intracranianos em pacientes encaminhados para estudos por tomografia de coerência óptica como portadores de glaucoma sem hipertensão ocular: relato de dois casos Intracranial tumors in patients referred for optical coherence tomography examination as glaucoma suspects: case report

    Directory of Open Access Journals (Sweden)

    Laurentino Biccas Neto

    2009-10-01

    Full Text Available A tomografia de coerência óptica (OCT tem se mostrado muito útil na avaliação de pacientes com glaucoma. São relatadas duas pacientes referidas com a suspeita de glaucoma sem hipertensão para avaliação por tomografia de coerência óptica que, na verdade, eram portadoras de tumores intracranianos - um cordoma de clivo no primeiro caso e um craniofaringeoma no segundo. Os achados à tomografia de coerência óptica - diminuição difusa da espessura da camada de fibras nervosas circumdiscais desproporcionalmente acentuada nos setores nasal e temporal - levantaram a suspeita de acometimento na região do quiasma e permitiram o diagnóstico destes importantes tumores intracranianos.Optical coherence tomography (OCT has proved to be a very valuable tool in the assessment of patients with glaucoma. In this report, intracranial tumors were discovered in two glaucoma suspects referred for diagnostic confirmation by OCT - a clivus chordoma and a craniopharyngeoma. Optical coherence tomography findings - marked asymmetrical diffuse attenuation of the peripapillary nerve fiber layer in nasal and temporal sectors - raised concerns about lesions in chiasmatic region and permitted the timely diagnosis of these intraocular tumors.

  20. Target-driven exploratory study of imatinib mesylate in children with solid malignancies by the Innovative Therapies for Children with Cancer (ITCC) European Consortium.

    Science.gov (United States)

    Geoerger, Birgit; Morland, Bruce; Ndiaye, Anna; Doz, Francois; Kalifa, Gabriel; Geoffray, Anne; Pichon, Fabienne; Frappaz, Didier; Chatelut, Etienne; Opolon, Paule; Hain, Sharon; Boderet, Francoise; Bosq, Jacques; Emile, Jean-Francois; Le Deley, Marie-Cecile; Capdeville, Renaud; Vassal, Gilles

    2009-09-01

    To explore imatinib efficacy and pharmacokinetics in children and adolescents with refractory/relapsing solid tumours, expressing imatinib-sensitive receptor tyrosine kinases. Exploratory study on imatinib in tumours expressing, at least, one of the receptors KIT or platelet-derived growth factor receptor (PDGFR). Standard radiological response evaluation, pharmacokinetics, gene mutations and positron emission tomography imaging were assessed. Thirty-six patients (median age: 13.7 years) with brain (12), mesenchymal/bone (14) or other solid tumours, received imatinib 340 mg/m(2)/d over a total of 255 months. Fifteen tumours expressed KIT in 30% cells, 19 expressed PDGFRA and 25 expressed PDGFRB. Twenty patients experienced grades 1-2 treatment-related toxicities. Ten patients achieved stable disease; one chordoma had metabolic response. Pharmacokinetic data showed high inter-patient variability (variation coefficient: 44% and 53% for plasma imatinib and CGP 74588 AUCs, respectively). Imatinib was tolerated well, but failed to show efficacy according to standard criteria in paediatric malignancies expressing KIT or PDGFR.

  1. Evaluation of a pencil-beam dose calculation technique for charged particle radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Petti, P.L. [Univ. of California, San Francisco, CA (United States)

    1996-07-15

    The purpose of this article is to evaluate a pencil-beam dose calculation algorithm for protons and heavier charged particles in complex patient geometries defined by computed tomography (CT) data and to compare isodose distributions calculated with the new technique to those calculated with conventional algorithms in selected patients with skull-base tumors. Monte Carlo calculations were performed to evaluate the pencil-beam algorithm in patient geometries for a modulated 150-MeV proton beam. A modified version of a Monte Carlo code described in a previous publication (18) was used for these comparisons. Tissue densities were inferred from patient CT data on a voxel-by-voxel basis, and calculations were performed with and without tissue compensators. A dose calculation module using the new algorithm was written, and treatment plans using the new algorithm were compared to plans using standard ray-tracing techniques for 10 patients with clival chordoma and three patients with nasopharyngeal carcinoma were treated with helium ions at Lawrence Berkeley National Laboratory (LBL). Pencil beam calculations agreed well with Monte Carlo calculations in the patient geometries. 23 refs., 5 figs.

  2. Neuropsychological function in adults after high dose fractionated radiation therapy of skull base tumors

    International Nuclear Information System (INIS)

    Glosser, Guila; McManus, Pat; Munzenrider, John; Austin-Seymour, Mary; Fullerton, Barbara; Adams, Judy; Urie, Marcia M.

    1997-01-01

    Purpose: To evaluate the long term effects of high dose fractionated radiation therapy on brain functioning prospectively in adults without primary brain tumors. Methods and Materials: Seventeen patients with histologically confirmed chordomas and low grade chondrosarcomas of the skull base were evaluated with neuropsychological measures of intelligence, language, memory, attention, motor function and mood following surgical resection/biopsy of the tumor prior to irradiation, and then at about 6 months, 2 years and 4 years following completion of treatment. None received chemotherapy. Results: In the patients without tumor recurrence or radiation necrosis, there were no indications of adverse effects on cognitive functioning in the post-acute through the late stages after brain irradiation. Even in patients who received doses of radiation up to 66 Cobalt Gy equivalent through nondiseased (temporal lobe) brain tissue, memory and cognitive functioning remained stable for up to 5 years after treatment. A mild decline in psycho-motor speed was seen in more than half of the patients, and motor slowing was related to higher radiation doses in midline and temporal lobe brain structures. Conclusion: Results suggest that in adults, tolerance for focused radiation is relatively high in cortical brain structures

  3. Decisions and involvement of cancer patient survivors: a moral imperative

    Directory of Open Access Journals (Sweden)

    Pravettoni G

    2016-12-01

    Full Text Available Gabriella Pravettoni,1,2 Ilaria Cutica,1,2 Simona Righetti,1 Ketti Mazzocco1,2 1Department of Oncology and Hematology, University of Milan, 2Applied Research Division for Cognitive and Psychological Science, European Institute of Oncology, Milan, Italy Purpose: The aim of this study was to review the experiences of direct involvement in patient survivorship for treatment and research. Methods: This is a narrative-focused review of the following two recent experiences of patient involvement: the Chordoma Foundation and the Triple Negative Breast Cancer Foundation. Results: These two examples represent concrete experiences that patients have built to favor a real involvement in the care and treatment of tumors. These experiences are profoundly modifying how cancer research is conducted and draw attention to the psychosocial dimensions of health care. Conclusion: These examples represent the new scenario in which modern medicine faces completely new challenges, copes with new needs, and cooperates with new health care professionals. Implications: Involving patients in a new perspective raises practical and ethical challenges for organizations to work together, for health providers to be professionally skilled and for the government to promote safeguarding policies. Keywords: patient empowerment, patients’ association, empowerment, skills, codesign ­techniques, cancer

  4. Assessment of the cervical spine denticulate ligament using MRI volumetric sequence: Comparison between 1.5 Tesla and 3.0 Tesla.

    Science.gov (United States)

    Seragioli, Rafael; Simao, Marcelo Novelino; Simao, Gustavo Novelino; Herrero, Carlos Fernando P S; Nogueira-Barbosa, Marcello H

    2018-03-01

    Denticulate ligaments (DLs) are pial extensions on each side of the spinal cord, comprising about 20 to 21 pairs of fibrous structures connecting the dura mater to the spinal cord. These ligaments are significant anatomical landmarks in the surgical approach to intradural structures. To our knowledge, there is no previous study on the detection of DLs using MRI. After IRB approval, we retrospectively evaluated 116 consecutive MRI scans of the cervical spine, using the volumetric sequence 3D COSMIC, 65 and 51 studies with 1.5T and 3.0T respectively. We did not include trauma and tumor cases. Two independent radiologists assessed the detection of cervical spine DLs independently and blinded for each cervical vertebral level. We compared the frequency of detection of these ligaments in 1.5 Tesla and 3.0 Tesla MRI using Fisher exact test considering P<0.05 as significant. We evaluated interobserver agreement with Kappa coefficient. We observed high detection frequency of the cervical spine DLs using both 1.5T (70 to 91%) and 3.0T (68 to 98%). We found no statistically significant difference in the detection frequency of ligaments between the 1.5T and 3.0T MRI in all vertebral levels. Using 3.0T, radiologists identified ligaments better in higher vertebral levels than for lower cervical levels (P=0.0003). Interobserver agreement on the identification of DL was poor both for 1.5T (k=0.3744; CI 95% 0.28-0.46) and 3.0T (k=0.3044; CI 95% 0.18-0.42) MRI. Radiologists identified most of the cervical DLs using volumetric MRI acquisition. Our results suggest 1.5T and 3.0T MRI performed similarly in the detection of DLs. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  5. NRASQ61K mutated primary leptomeningeal melanoma in a child: case presentation and discussion on clinical and diagnostic implications

    International Nuclear Information System (INIS)

    Angelino, Giulia; De Pasquale, Maria Debora; De Sio, Luigi; Serra, Annalisa; Massimi, Luca; De Vito, Rita; Marrazzo, Antonio; Lancella, Laura; Carai, Andrea; Antonelli, Manila; Giangaspero, Felice; Gessi, Marco; Menchini, Laura; Scarciolla, Laura; Longo, Daniela; Mastronuzzi, Angela

    2016-01-01

    Primary melanocytic neoplasms are rare in the pediatric age. Among them, the pattern of neoplastic meningitis represents a peculiar diagnostic challenge since neuroradiological features may be subtle and cerebrospinal fluid analysis may not be informative. Clinical misdiagnosis of neoplastic meningitis with tuberculous meningitis has been described in few pediatric cases, leading to a significant delay in appropriate management of patients. We describe the case of a child with primary leptomeningeal melanoma (LMM) that was initially misdiagnosed with tuberculous meningitis. We review the clinical and molecular aspects of LMM and discuss on clinical and diagnostic implications. A 27-month-old girl with a 1-week history of vomiting with mild intermittent strabismus underwent Magnetic Resonance Imaging, showing diffuse brainstem and spinal leptomeningeal enhancement. Cerebrospinal fluid analysis was unremarkable. Antitubercular treatment was started without any improvement. A spinal intradural biopsy was suggestive for primary leptomeningeal melanomatosis. Chemotherapy was started, but general clinical conditions progressively worsened and patient died 11 months after diagnosis. Molecular investigations were performed post-mortem on tumor tissue and revealed absence of BRAF V600E , GNAQ Q209 and GNA11 Q209 mutations but the presence of a NRAS Q61K mutation. Our case adds some information to the limited experience of the literature, confirming the presence of the NRAS Q61K mutation in children with melanomatosis. To our knowledge, this is the first case of leptomeningeal melanocytic neoplasms (LMN) without associated skin lesions to harbor this mutation. Isolated LMN presentation might be insidious, mimicking tuberculous meningitis, and should be suspected if no definite diagnosis is possible or if antitubercular treatment does not result in dramatic clinical improvement. Leptomeningeal biopsy should be considered, not only to confirm diagnosis of LMN but also to study

  6. The importance of surgery as part of multimodal therapy in rapid progressive primary extraosseous Ewing sarcoma of the cervical intra- and epidural space

    Directory of Open Access Journals (Sweden)

    Richard Bostelmann

    2016-12-01

    Full Text Available Primary extraosseous Ewing sarcomas (EESs are an extremely rare pathological entity. Less than 32 cases have been reported in the literature. Here we report an uncommon case with very rapid progression in the cervical region with extra- and intradural involvement. We present a thorough review of the literature and discuss possible treatment modalities. The Medline database was searched using the search terms: Ewing sarcoma, extraosseus tumour, treatment, management, cervical spine. A previously healthy 29-year-old man complained of right-sided radiculopathy (C7. Magnetic resonance imaging showed an enhancing foraminal, sandglass shaped neurinoma- like lesion. Surgery revealed an intraand extra-dural lesion, which was histologically diagnosed as Ewing sarcoma. Despite gross total resection, there was a massive symptomatic tumor recurrence within 6 weeks. A second gross total resection was realized. The patient was treated according to the EURO E.W.I.N.G.-Protocol (VIDE and recovered very well (progression-free interval during therapy. Several decompressive re-surgeries were realized with adjuvant radio-chemotherapy. At the last follow-up (17 months after initial surgery the patient was in remission with a good quality of live. This case is to illustrate that despite extensive therapeutic efforts, the progression- free survival in case of primary EES may be very short. To maintain neurological function and good quality of live as long as possible, a multimodal strategy seems to be adequate. Like in the present case this implies several surgeries and adjuvant chemo-and radiotherapy. Whether this improves overall survival remains unclear.

  7. Circumferential dural resection technique and reconstruction for the removal of giant calcified transdural herniated thoracic discs.

    Science.gov (United States)

    Walker, Corey T; Kalani, M Yashar S; Oppenlander, Mark E; Godzik, Jakub; Martirosyan, Nikolay L; Standerfer, Robert J; Theodore, Nicholas

    2018-02-01

    OBJECTIVE The authors report a novel paradigm for resection of the disc or dural complex to treat giant calcified transdural herniated thoracic discs, and they describe a technique for the repair of dural defects. These herniated thoracic discs are uncommon, complicated lesions that often require a multidisciplinary team for effective treatment. The intradural component must be removed to effectively decompress the spinal cord. The opening of the friable dura mater, which frequently adheres to the extradural component of the disc, can result in large defects and difficult-to-manage CSF leaks. METHODS The authors performed a retrospective study of the technique and outcomes in patients with a transdural herniated disc treated at St. Joseph's Hospital and Medical Center within a 4-year period between 2012 and 2015. RESULTS During the study period, 7 patients (mean age 56.1 years) presented to the department of neurosurgery with clinical symptoms consistent with myeloradiculopathy. In all cases, 2-level corpectomies of the involved levels were combined with circumferential resection of the dura and complete decompression of the spinal cord. The dural defect was repaired with an onlay dural patch, and a large piece of AlloDerm (LifeCell Corp) graft was sewn to close the pleural defect. Every patient had a perioperative lumbar drain placed for CSF diversion. No patient suffered neurological decline related to the surgery, and 3 patients experienced clinically significant improvement in function. Two patients developed an early postoperative CSF leak that required operative revision to oversew the defects. CONCLUSIONS This novel technique for decompression of the spinal cord by dural resection for the removal of giant calcified transdural herniated thoracic discs is safe and results in excellent decompression of the spinal cord. The technique becomes necessary when primary repair of the dura is not possible, and it can be used in cases in which the resection of pathology

  8. Pediatric intraspinal neoplasms; Paediatrische intraspinale Neoplasien

    Energy Technology Data Exchange (ETDEWEB)

    Mueller, U.; Ahlhelm, F. [Kantonsspital Baden AG, Institut fuer Radiologie, Baden (Switzerland); Ulmer, S. [Medizinisch-Radiologisches Institut, Zuerich (Switzerland); Schlaeger, R. [Universitaetsspital Basel, Neurologische Klinik und Poliklinik, Basel (Switzerland); UCSF, Department of Neurology, San Francisco (United States)

    2014-08-15

    With an overall incidence of 10 % of all central nervous system tumors, spinal tumors are relatively rare in children. The majority of these tumors are astrocytomas and ependymomas (70 %) followed by rare non-glial tumor entities, such as gangliogliomas. They can be differentiated into intramedullary, extramedullary intradural and extramedullary extradural tumors according to their occurrence within the anatomical intraspinal compartments. The clinical presentation is generally unspecific. Longer lasting back pain or a gradually worsening scoliosis are often the first signs of the disease. Neurological deficits, such as gait disturbances and paresis often occur after a time delay. In rare cases increased intracranial pressure has been reported. Knowledge concerning potential organ manifestations, resulting complications and typical radiological presentation, especially in magnetic resonance imaging are mandatory for adequate diagnosis and treatment of affected patients. (orig.) [German] Spinale Tumoren umfassen bei Kindern etwa 10 % aller auftretenden Tumoren des Zentralnervensystems (ZNS) und sind damit relativ selten. Die ueberwiegende Mehrheit dieser Tumoren sind Astrozytome und Ependymome (70 %), gefolgt von selteneren nichtglialen Tumorentitaeten, wie z. B. Gangliogliomen. Differenziert werden koennen sie nach ihrem Auftreten innerhalb der anatomischen intraspinalen Kompartimente in intra- und extramedullaere sowie intra- und extradurale Tumoren. Ihre klinische Praesentation ist haeufig unspezifisch. Laenger bestehende Rueckenschmerzen oder eine progrediente Skoliose sind oft erste Hinweise auf eine Erkrankung. Neurologische Ausfallerscheinungen wie Gangstoerungen und progrediente Laehmungen treten meist zeitlich verzoegert auf. In Einzelfaellen wird ueber Hirndruckzeichen berichtet. Kenntnisse ueber die moegliche Organmanifestation, resultierende Komplikationen und die typischen radiologischen Befunde v. a. in der MRT sind entscheidend fuer die adaequate

  9. High lumbar disc herniation coexisting with thoracic meningioma – an issue of differential diagnostic

    Directory of Open Access Journals (Sweden)

    Dabija M.

    2017-09-01

    Full Text Available Meningiomas involving the spinal compartment are relatively rare compared to the intracranial ones representing between 7,5% and 12,5% of all CNS meningiomas, most of them being located in the intradural compartment respecting the pial layer of the spinal cord. Affecting ten times more often women rather than men, especially in the 5th and 6th decade of life, they pose a difficult diagnostic setting despite modern neuroimagistic techniques. The matter of correct differential diagnosis becomes even more doubtful, most of the patients of that age having also associative degenerative disorders, e.g. lumbar disc herniation. In this case proper neurological examination is the key as we will furthermore demonstrate - the MRI exam being targeted on the level of the sensorial dysfunction and not on the most common region which gives according pathology. The following case presentation can be considered “classic” representing a patient with all the criteria mentioned above including a long history of rheumatologic and chronic back pain medication for pain relief. We used the Frankel Scale for grading the pre- and postoperative neurological status. Acute neurological deterioration (under 24 hours is an important criteria for admission in emergency unit with around the clock exploration followed by microsurgical operation for the best possible outcome. Coexisting chronic lesions may be left behind and kept under surveillance, operated only when clear signs of health disorder appear. Because there are reported cases of thoracic meningiomas discovered after hernia disc operation and even cases of paraplegia due to a missed lesion, the aim of this article is to emphasize the importance of proper neurological examination preceding any MRI exploration.

  10. A review article on the diagnosis and treatment of cerebrospinal fluid fistulas and dural tears occurring during spinal surgery.

    Science.gov (United States)

    Epstein, Nancy E

    2013-01-01

    In spinal surgery, cerebrospinal fluid (CSF) fistulas attributed to deliberate dural opening (e.g., for tumors, shunts, marsupialization of cysts) or inadvertent/traumatic dural tears (DTs) need to be readily recognized, and appropriately treated. During spinal surgery, the dura may be deliberately opened to resect intradural lesions/tumors, to perform shunts, or to open/marsupialize cysts. DTs, however, may inadvertently occur during primary, but are seen more frequently during revision spinal surgery often attributed to epidural scarring. Other etiologies of CSF fistulas/DTs include; epidural steroid injections, and resection of ossification of the posterior longitudinal ligament (OPLL) or ossification of the yellow ligament (OYL). Whatever the etiology of CSF fistulas or DTs, they must be diagnosed utilizing radioisotope cisternography (RIC), magnetic resonance imaging (MRI), computed axial tomography (CT) studies, and expeditiously repaired. DTs should be repaired utilizing interrupted 7-0 Gore-Tex (W.L. Gore and Associates Inc., Elkton, MD, USA) sutures, as the suture itself is larger than the needle; the larger suture occludes the dural puncture site. Closure may also include muscle patch grafts, dural patches/substitutes (bovine pericardium), microfibrillar collagen (Duragen: Integra Life Sciences Holdings Corporation, Plainsboro, NJ), and fibrin glues or dural sealants (Tisseel: Baxter Healthcare Corporation, Deerfield, IL, USA). Only rarely are lumbar drains and wound-peritoneal and/or lumboperitoneal shunts warranted. DTs or CSF fistulas attributed to primary/secondary spinal surgery, trauma, epidural injections, OPLL, OYL, and other factors, require timely diagnosis (MRI/CT/Cisternography), and appropriate reconstruction.

  11. The use of recombinant nAG protein In spinal cord crush injury in a rat model

    International Nuclear Information System (INIS)

    Al-Qattan, M.M.; Al-Motairi, M.; Ah-Habib, A.

    2017-01-01

    Objective: To evaluate the therapeutic properties of nAG protein during the recovery following acute spinal cord injuries in the rat. Study Design: An experimental study. Place and Duration of Study: King Saud University, Riyadh, Saudi Arabia, from September 2014 to September 2015. Methodology: Eight rats were studied (4 control rats and 4 experimental rats; and hence 50% were controls and 50% were experimental). All rats were subjected to an acute spinal cord injury using the aneurysmal clip injury model. Immediately after the injury, a single intra-dural injection of either normal saline (in the control group) or the nAG protein (in the experimental group) was done. Assessment of both groups was done over a 6-week period with regard to weight maintenance, motor recovery scores, MRI and histopathology of the injury site. Results: Weight maintenance was seen in the experimental and not in the control rats. Starting at 3 weeks after injury, the motor recovery was significantly (p<0.05) better in the experimental group. MRI assessment at 6 weeks showed better maintenance of cord continuity and less fluid accumulation at the injury site in the nAG-treated group. Just proximal to the injury site, there was less gliosis in the experimental group compared to the control group. At the crush injury site, there was less tissue architecture distortion, less vacuole formation, and less granulation tissue formation in the experimental group. Conclusion: The local injection nAG protein enhances neuro-restoration, reduces gliosis, and reduces vacuole/ granulation tissue formation following acute spinal cord crush injury in the rat aneurysmal clip animal model. (author)

  12. Diffusion tensor tractography of normal and compressed spinal cord: a preliminary study at 3.0 T MR

    International Nuclear Information System (INIS)

    Wang Wei; Chang Shixin; Hao Nanxin; Du Yushan; Wang Yibin; Zong Genlin; Cao Kaiming; Lu Jianping; Zhao Cheng; Qin Wen

    2007-01-01

    Objective: To study the feasibility and clinical values of diffusion tensor tractography (DTT) in the spinal cord at 3.0 T MR. Methods: Forty patients with spinal cord compression including cervical cord herniation and cervical spondylosis (30 cases), tumors in spinal canal (9 cases) and old injury in cervical vertebrae (1 cases) and 20 healthy volunteers participated in this study. Single-shot spin- echo echo-planar diffusion tensor sequence for tractography of the spinal cord was performed. The fibers of spinal cord were visualized by using fiber tracking software. Results: On the DTT maps, the normal spinal cord was depicted as a fiber tract showing color-encoded cephalocaudally, which indicated anisotropy in the cephalocaudal direction. By setting two ROI, the main spinal cord fiber tracts, such as corticospinal or spinothalamic tract, were visualized. The tracts from two sides of the brain did not completely cross. It was asymmetric in the number of tracts on the two sides in most normal subjects (8/10). The tracts of all patients with cord compression were seen oppressed or damaged in different degrees. The DTT in patients with cervical spondylosis and extramedullary-intradural neurolemmoma demonstrated that tracts were oppressed but not damaged. The DTT in one ependymoma showed that tract was markedly compressed and slightly damaged. Conclusion: DTT is a promising tool for demonstrating the spinal cord tracts and abnormalities, can provide useful information for the localization of compression and evaluation of the impairment extent on the white matter tracts of the spinal cord. (authors)

  13. Fatty filum terminale (FFT) as a secondary tethering element in children with closed spinal dysraphism.

    Science.gov (United States)

    Gupta, Ankush; Rajshekhar, Vedantam

    2017-12-19

    The purpose of this study was to assess the prevalence of FFT as an additional tethering element in children operated for closed spinal dysraphism, where FFT was not the primary tethering pathology. This is a retrospective study of 195 children (dermal sinus (n = 28, 14.4%), and dermoid cyst (n = 10, 5.1%). Factors such as age and sex, presenting symptoms, intraoperative findings, and radiological presence of a FFT on a magnetic resonance imaging (MRI) were documented, and the relationship between the primary diagnoses and presence of FFT was analyzed. FFT as a secondary finding was seen in 63 patients (32.3%). The mean age of the cohort was 54 months (4.5 years) and the sex distribution was relatively even (51.8% girls). The commonest symptom at presentation was a swelling in the back, followed by lower limb weakness. The mean duration of symptoms was nearly 30 months. FFT was seen on the MRI and confirmed intraoperatively in 55 patients (28.2%). There were 8 patients (4.1%) where a FFT was seen intraoperatively, but was not diagnosed on the preoperative MRI. In 16 patients, FFT was seen > 2 segments away from the primary tethering pathology, 8 of which mandated a second skin incision for sectioning of the FFT. Secondary FFT was most commonly associated with a SCM (types I and II combined) and was seen in 42.6% of those patients. It was least commonly associated with intradural dermoid cysts. The presence of a secondary FFT should be considered and actively sought on preoperative thin-slice T1W axial MR images in the sacral region in all patients with spinal dysraphism. Even if a FFT is not seen on preoperative MR images, the filum should be explored and sectioned if it is in the vicinity of the primary surgical field, especially in patients with SCM.

  14. Spinal lipomatous malformations.

    Science.gov (United States)

    Jindal, A; Mahapatra, A K

    2000-05-01

    Spinal lipomatous malformations (SLM) include all the closed neural tube defects (NTD) with excessive lipomatous tissue in the spinal cord or filum terminale. We evaluated 65 cases of SLM seen & operated at our department in the last 7 years. Of these only 9 were asymptomatic and 8 were below 2 years of age. There were more males than females. In addition to subcutaneous lipoma many patients also had hypertrichiosis and dermal sinus as cutaneous makes. Twenty patients had foot deformity and 5 had unilateral limb shortening. Sixty-two patients had MRI and 3 had CT myelogram for evaluation. These revealed 7 patients with Chiari malformations, 10 with focal syrinx and 2 patients underwent VP shunt for hydrocephalus. Sacral agenesis was seen in 5 patients. Clinical features were similar to other cases of spinal dysraphism except that sensory loss and trophic ulcer were more frequent. Intradural lipoma and tethering was seen in 18 cases whereas intramedullary lipoma & conus lipoma was seen in 40 & 7 cases respectively. Additional tethering lesion was seen in 1/3 cases and was treated simultaneously. Preoperative deficits improved in 28 cases and stabilized in 33 cases. Three patients developed fresh deficits after surgery. We observed 8 CSF leaks and 4 wound infections in postoperative period. It is very clear from our data that a patient has about 95% chances that his neurological status may improve or stabilize following surgery and the risk of developing fresh deficits is about 5%. We, therefore, suggest that all patients of SLM should be treated with aggressive surgical management for best results.

  15. Surgery and subsequent rehabilitation for cervical spine tumours compressing neural structures.

    Science.gov (United States)

    Kiwerski, Jerzy E

    2008-01-01

    Bone malignancies account for merely about 1.5% of all cancers, with a small percentage of these tumours developing in the cervical spine. However, the cervical spine is also the site of benign tumours and neoplasms involving not only bony tissue. Benign tumours do not metastasize but pose a threat to the spinal cord when located intrathecally. Even though such tumours do not represent malignancy, they are considered to be locally malignant. The most common cervical spine neoplasms are intradural tumours, usually extramedullary: neurofibromas, meningiomas or gliomas.Indications for surgery depend of the nature and location of the tumour and the consequences of tumour growth. Surgery is obviously necessary for intrathecal tumours compressing the spinal cord. The choice of surgical approach and manner of stabilisation depend primarily on the location of the lesion and the presence of spinal cord compression.Rehabilitation is indicated in all patients, but is particularly important, and at the same time difficult, when the growth of the tumour has resulted in neurological disturbances. The task is all the more difficult when in the presence of a massive and high spinal cord damage. Rehabilitation programmes should be designed individually for each patient and should account for the degree of paresis, stage of the underlying malignant disease, survival prognosis, disturbances in the function of other systems, apart from musculoskeletal apparatus, age of the patient, his or her commitment to treatment and other factors.The treatment of malignant neoplasms is usually associated with an unfavourable outcome. However, combination drug treatments, radiation therapy and surgery with subsequent rehabilitation will often prolong survival, ameliorate suffering and improve patients' quality of life.

  16. Utility of the Lateral Base Dural Tacking Method in Cord Tumor Surgery Performed Using Unilateral Hemilaminectomy: A Comparison of Dural Window Widths.

    Science.gov (United States)

    Lee, Seong-Jong; Im, Soo Bin; Jeong, Je Hoon; Chung, Moonyoung; Kim, Bum-Tae; Hwang, Sun-Chul; Shin, Dong-Seong

    2018-02-01

    Unilateral hemilaminectomy, which is used to remove spinal cord tumors, is simpler than laminoplastic laminotomy and affords certain biomechanical advantages. However, both incomplete tumor removal and inadvertent infliction of spinal cord damage attributable to the narrow surgical corridor remain of concern. When a spinal cord tumor is to be removed, it is important to ensure that the dural window along the surgical corridor is of adequate width. This study aimed to determine that the utility of lateral base dural tacking (LBT) method when cord tumor surgery is performed using a unilateral hemilaminectomy-a comparison of dural window widths with a traditional dural tack-up and a suspending-out (DSO) method with the aid of digital image-analysis software. Twenty-one consecutive patients who had intradural-extramedullary spinal cord tumors removed using a unilateral hemilaminectomy were included in the study and analyzed retrospectively. We acquired DSO and LBT dural window images using surgical microscopes under identical conditions in consecutive order and then removed the tumors using the LBT method. We used digital image-analysis software to analyze the images quantitatively. The pixel numbers of LBT and DSO window were compared using a paired t test. Twenty-one tumorous lesions were successfully removed without any major problems using a unilateral hemilaminectomy through LBT windows. The mean pixel numbers of the LBT and DSO windows were 126,787 ± 41,938 and 85,940 ± 21,638. The LBT windows were 46% larger than the DSO windows (P < 0.001). We objectively proved that the utility of the LBT method for widening the surgical corridor created during hemilaminectomy. Copyright © 2017 Elsevier Inc. All rights reserved.

  17. Age related outcome in acute subdural haematoma following traumatic head injury.

    LENUS (Irish Health Repository)

    Hanif, S

    2009-09-01

    Acute subdural haematoma (ASDH) is one of the conditions most strongly associated with severe brain injury. Reports prior to 1980 describe overall mortality rates for acute subdural haematomas (SDH\\'s) ranging from 40% to 90% with poor outcomes observed in all age groups. Recently, improved results have been reported with rapid diagnosis and surgical treatment. The elderly are predisposed to bleeding due to normal cerebral atrophy related to aging, stretching the bridging veins from the dura. Prognosis in ASDH is associated with age, time from injury to treatment, presence of pupillary abnormalities, Glasgow Coma Score (GCS) or motor score on admission, immediate coma or lucid interval, computerized tomography findings (haematoma volume, degree of midline shift, associated intradural lesion, compression of basal cisterns), post-operative intracranial pressure and type of surgery. Advancing age is known to be a determinant of outcome in head injury. We present the results of a retrospective study carried out in Beaumont Hospital, Dublin, Ireland\\'s national neurosurgical centre. The aim of our study was to examine the impact of age on outcome in patients with ASDH following severe head injury. Only cases with acute subdural haematoma requiring surgical evacuation were recruited. Mortality was significantly higher in older patients (50% above 70 years, 25.6% between 40 and 70 years and 26% below 40 years). Overall poor outcome (defined as Glasgow outcome scores 3-5) was also higher in older patients; 74.1% above 70 years, 48% between 40 and 70 years and 30% below 40 years. Poor outcome in traumatic acute subdural haematoma is higher in elderly patients even after surgical intervention.

  18. Epidural interlaminar injections in severe degenerative lumbar spine: fluoroscopy should not be a luxury.

    Science.gov (United States)

    Filippiadis, Dimitrios K; Rodt, Thomas; Kitsou, Maria-Chrysanthi; Batistaki, Chrysanthi; Kelekis, Nikolaos; Kostopanagiotou, Georgia; Kelekis, Alexis

    2017-09-12

    To assess technical efficacy, accuracy, and safety of epidural (interlaminar) injections performed blindly in patients with a severely degenerated lumbar spine. Over 12 consecutive months, 138 patients with a severe degenerative lumbar spine underwent epidural (interlaminar) injection as therapy for low back pain and neuralgia. Patients had already undergone a blind epidural infiltration with minimum or no pain reduction. The session was repeated in the angiography suite. Patients were placed in the lateral decubitus position. The injection was performed without image guidance by an anaesthesiologist; the target level was defined before the beginning of the procedure. Once air resistance loss was felt it was presumed that the needle was inside the epidural space. Verification of needle position was performed by injection of 1-3 mL of iodinated contrast medium under fluoroscopy in a lateral projection. Correct needle position inside the epidural space was documented in 82/138 cases (59.4%); unexpected extraepidural location was seen in 56/138 cases (40.6%). Target level was reached in 96/138 cases (69.6%); in 42/138 cases (30.4%) the needle was positioned in a non-target level. In 5/138 (3.6%) cases, there was inadvertent intradural position of the needle. Image guidance was subsequently used for correct positioning of the needle, which was feasible in all cases. Blind interlaminar epidural injections lack the accuracy of exact needle location that imaging guidance offers in approximately 40% of cases, when there is difficult spine anatomy and the initial epidural approach has failed to provide pain relief. Image guidance for interlaminar epidural injection ensures accurate needle placement, enhancing the safety and efficacy of the procedure. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  19. Tumores dermóides e epidermóides intra-espinhas

    Directory of Open Access Journals (Sweden)

    Oscar Fontenelle Filho

    1971-03-01

    Full Text Available São relatados dois casos de tumores epidermóides e um de tumor dermóide, todos intrarraquianos. Este último era de localização epidural ao nível da coluna torácica (caso 3; os dois tumores epidermóides situavam-se na coluna tóraco-lombar (caso 1 e lombar (caso 2, respectivamente, sendo o primeiro intramedular e o segundo intradural. Em dois casos (casos 2 e 3 os tumores associavam-se a fístula dérmica congênita. Um paciente (caso 3 foi operado aos dois meses de idade; a descoberta do tumor deveu-se à realização da raquimanometria que revelou bloqueio, apesar do paciente não apresentar qualquer sinal neurológico de compressão medular. Os autores são de opinião que, em presença de fístula dérmica congênita ao nível da coluna vertebral, principalmente quando localizada acima do segmento lombosacro, deve-se sempre suspeitar da possibilidade do tumor epidermóide ou dermóide intrarraquiano, mesmo na ausência de sinais neurológicos. A combinação de sintomas neurológicos de longa duração, a evidência radiológica de erosão e alargamento do canal raquiano e a história de fístula dérmica congênita proporcionaram o diagnóstico pré-operatório correto no caso 2.

  20. Disseminated primary diffuse leptomeningeal gliomatosis: a case report with liquid based and conventional smear cytology

    Directory of Open Access Journals (Sweden)

    Bilic Masha

    2005-09-01

    Full Text Available Abstract Background Primary diffuse leptomeningeal gliomatosis is a rare neoplasm confined to the meninges without evidence of primary tumor in the brain or spinal cord parenchyma. Cerebrospinal fluid diversion via ventriculoperitoneal shunt may be used as a therapeutic modality. Herein, we describe the first report of cytologic findings of a case of this neoplasm with shunt-related peritoneal metastasis. Case presentation A 19-year-old male presented with a 6-month history of severe headaches. He had bilateral papilledema on physical exam. Cerebrospinal fluid examination was negative. Four months later a ventriculoperitoneal shunt was placed. Shortly thereafter, he was diagnosed with primary diffuse leptomeningeal gliomatosis based on the biopsy of an intradural extramedullary lesion adjacent to the lumbar spinal cord at a referral cancer center. The histology featured an infiltrating growth pattern of pleomorphic astrocytes with diffuse positivity for glial fibrillary acidic protein. A couple of months later he presented at our institution with ascites and an anterior peritoneal mass. Repeat cerebrospinal fluid cytology and fine needle aspiration of the mass confirmed disseminated gliomatosis. Cytologic characteristics included clusters of anaplastic cells of variable size, high nuclear to cytoplasm ratio and scant to moderate cytoplasm. Occasional single bizarre multinucleated cells were seen with eccentric "partial wreath-like" nuclei, clumped chromatin and prominent nucleoli. Patient expired 13 months after initial presentation. Conclusion Disseminated primary diffuse leptomeningeal gliomatosis should be considered in the differential diagnosis of chronic aseptic meningitis and in the presence of a peritoneal tumor in patients with ventriculoperitoneal shunts. Immunocytochemistry may be of diagnostic value.

  1. Tratamiento Quirúrgico de los Meningiomas del Foramen Óptico, Técnicay Resultados de una Serie de 18 Pacientes

    Science.gov (United States)

    Goldschmidt, Ezequiel; Ajler, Pablo; Campero, Álvaro; Landriel, Federico; Sposito, Maximiliano; Carrizo, Antonio

    2014-01-01

    Introducción: los meningiomas del foramen óptico producen un rápido deterioro de la función visual aún cuando su tamaño es pequeño, por eso su diagnóstico y manejo difiere del resto de los meningiomas clinoideos. El propósito de este estudio es presentar la técnica y los resultados de nuestro manejo quirúrgico de meningiomas foraminales (MF). Pacientes y Métodos: se llevó a cabo una revisión de las historias clínicas de 47 pacientes con meningiomas primarios intraorbitarios. Se realizaron 52 cirugías en los pacientes con MF. Se empleó una craneotomía fronto-orbitaria, seguida de una descompresión extradural del canal óptico, resección del componente intraorbitario y exploración intradural del nervio óptico. Resultados: de los 12 pacientes con MF que presentaban la visión conservada, la agudeza visual fue preservada en 7 casos, mejoró en 2, y empeoró en 3. En 18 pacientes, el principal síntoma fue exoftalmos y en 35 pacientes ceguera unilateral. Ocurrieron 6 recurrencias, 2 a 10 años después de la resección quirúrgica. Cinco de ellos fueron reoperados. Se indicó radioterapia después de la recurrencia en 3 pacientes. Conclusión: el manejo de los MF continúa siendo controvertido y frecuentemente se propone un tratamiento conservador. Basados en nuestros hallazgos de frecuente extensión intracraneal, proponemos realizar una resección total o subtotal del tumor, preservando el nervio óptico en pacientes con visión prequirúrgica conservada. PMID:25165616

  2. First Case of Autonomic Dysreflexia Following Elective Lower Thoracic Spinal Cord Transection in a Spina Bifida Adult.

    Science.gov (United States)

    Garces, Juanita; Mathkour, Mansour; Scullen, Tyler; Kahn, Lora; Biro, Erin; Pham, Alex; Sulaiman, Olawale A R; Smith, Roger; Bui, Cuong J

    2017-12-01

    Spinal cord transection is a radical but effective treatment for highly selective cases of symptomatic spinal retethering in paraplegic spina bifida patients. Autonomic dysreflexia (AD) is a potentially life-threatening syndrome involving a dysregulated sympathetic discharge reflex commonly seen following cervical and high thoracic spinal cord injury, leading to a disconnect between autonomic pathways above and below the lesion that can lead to severe complications including uncontrolled hypertension, bradycardia, stroke, and potentially death. Herein we present a case in which a paraplegic spina bifida patient presenting with symptomatic spinal retethering experienced autonomic dysreflexia following an elective spinal cord transection. A 51-year-old male with a history of complex spina bifida presented with an active cerebrospinal fluid leak. Physical examination revealed a thin covering of abnormal epidermis over the large placode. Magnetic resonance imaging revealed a large myelomeningocele defect with posterior element defects spanning from L2 to the sacrum with evidence of tethering. The patient underwent an intradural transection of the spinal cord with a "blind-pouch" closure of the dura at the level of T12/L1. Postoperatively, the patient developed intermittent episodes of hypertension, bradycardia, headaches, altered mental status, severe perspiration, and red flushing of the upper torso, face, and arms. The diagnosis of AD was made clinically and managed with a positive response to a combination of beta- and alpha-blockade along with patient education on avoidance of common AD triggers. At 5-year follow-up the patient has continued to do well on medication. This case highlights a potential major side effect from elective transection of the spinal cord. If unrecognized and untreated, AD can cause significant distress and morbidity. We hope this first case report serves to supplement existing data and aid in future surgical and medical decision

  3. Magnetic resonance imaging of spinal tumors. A study using a 0.3 T vertical magnetic field

    International Nuclear Information System (INIS)

    Li Ming Hua.

    1992-12-01

    A total of 168 patients with spinal tumors were evaluated with MRI. The study shows that MRI is a sensitive method for demonstration of spinal tumors. MRI also provides a possibility to separate different histological types of tumors based on their morphology and signal characteristics. Intramedullary tumors (25 cases): Ependymomas (6 cases) and astrocytomas (7 cases) were most common. Ependymomas have a more irregular signal pattern than astrocytomas. Astrocytomas are more common in the upper spine and are more often completely cystic. Contrast enhancement is important for separation of cyst, edema and solid tumor. Intradural extramedullary tumors (31 cases): Neuromas (14 cases) and meningeomas (11 cases) were most common. Neuromas always had markedly increased signal intensity on T2-weighted images. Meningeomas were only hyperintense occasionally. Neuromas were more inhomogeneous than meningeomas on T1-weighted images. Contrast enhancement is valuable for delineation of small tumors. Extradural tumors (91 cases): 76 patients had metastases, 7 primary spinal tumors and 8 multiple myelomas. T1-weighted images are almost always superior to other sequences because tumor invasion in the fatty bone marrow is seen as a low signal area in contrast to the high signal from the fat. Spinal lymphomas (14 cases): May be divided into vertebral, paraspinal and epidural tumors. Most cases have all locations. Spinal neurofibromatosis (7 cases): Most patients had multiple, often bilateral neurofibromas. One patient had a meningeomas on one spinal dysplasia with meningoceles. MRI is superior to other modalities for evaluation of the full extent of the disease. The coronal view is often valuable because of the arrangement of the tumors. In addition to providing diagnosis, MRI is of great value in treatment follow-up

  4. Endovascular and surgical treatment of spinal dural arteriovenous fistulas

    International Nuclear Information System (INIS)

    Andres, Robert H.; University of Berne; Stanford University Medical Center, Department of Neurosurgery, Stanford, CA; University of Berne; Barth, Alain; Medical University of Graz, Department of Neurosurgery, Graz; University of Berne; Guzman, Raphael; Stanford University Medical Center, Department of Neurosurgery, Stanford, CA; University of Berne; Remonda, Luca; El-Koussy, Marwan; Schroth, Gerhard; University of Berne; Seiler, Rolf W.; Widmer, Hans R.; University of Berne

    2008-01-01

    The aim of this retrospective study was to evaluate the clinical outcome of patients with spinal dural arteriovenous fistulas (SDAVFs) that were treated with surgery, catheter embolization, or surgery after incomplete embolization. The study included 21 consecutive patients with SDAVFs of the thoracic, lumbar, or sacral spine who were treated in our institution from 1994 to 2007. Thirteen patients were treated with catheter embolization alone. Four patients underwent hemilaminectomy and intradural interruption of the fistula. Four patients were treated by endovascular techniques followed by surgery. The clinical outcome was assessed using the modified Aminoff-Logue scale (ALS) for myelopathy and the modified Rankin scale (MRS) for general quality of life. Patient age ranged from 44 to 77 years (mean 64.7 years). Surgical as well as endovascular treatment resulted in a significant improvement in ALS (-62.5% and -31.4%, respectively, p<0.05) and a tendency toward improved MRS (-50% and -32%, respectively) scores. Patients that underwent surgery after endovascular treatment due to incomplete occlusion of the fistula showed only a tendency for improvement in the ALS score (-16.7%), whereas the MRS score was not affected. We conclude that both endovascular and surgical treatment of SDAVFs resulted in a good and lasting clinical outcome in the majority of cases. In specific situations, when a secondary neurosurgical approach was required after endovascular treatment to achieve complete occlusion of the SDAVF, the clinical outcome was rather poor. The best first line treatment modality for each individual patient should be determined by an interdisciplinary team. (orig.)

  5. Endoscopic endonasal anatomy and approaches to the anterior skull base: a neurosurgeon's viewpoint.

    Science.gov (United States)

    Abuzayed, Bashar; Tanriover, Necmettin; Gazioglu, Nurperi; Sanus, Galip Zihni; Ozlen, Fatma; Biceroglu, Huseyin; Kafadar, Ali Metin; Eraslan, Berna Senel; Akar, Ziya

    2010-03-01

    The objective of this study was to review the endoscopic anatomy of the anterior skull base, defining the pitfalls of endoscopic endonasal approaches to this region. Recently, these approaches are gaining popularity among neurosurgeons, and the details of the endoscopic anatomy and approaches are highlighted from the neurosurgeons' point of view, correlated with demonstrative cases. Twelve fresh adult cadavers were studied (n = 12). We used Karl Storz 0 and 30 degrees, 4 mm, 18- and 30-cm rod lens rigid endoscope in our dissections. After preparation of the cadaveric specimens, we approached the anterior skull base by the extended endoscopic endonasal approach. After resection of the superior portion of the nasal septum, bilateral middle and superior turbinates, and bilateral anterior and posterior ethmoidal cells, we could obtain full exposure of the anterior skull base. The distance between optic canal and the posterior ethmoidal artery ranged from 8 to 16 mm (mean, 11.08 mm), and the distance between posterior ethmoidal artery and the anterior ethmoidal artery ranged from 10 to 17 mm (mean, 13 mm). After resecting the anterior skull base bony structure and the dura between the 2 medial orbital walls, we could visualize the olfactory nerves, interhemispheric sulcus, and gyri recti. With dissecting the interhemispheric sulcus, we could expose the first (A1) and second (A2) segments of the anterior cerebral artery, anterior communicating artery, and Heubner arteries. This study showed that extended endoscopic endonasal approaches are sufficient in providing wide exposure of the bony structures, and the extradural and intradural components of the anterior skull base and the neighboring structures providing more controlled manipulation of pathologic lesions. These approaches need specific skill and learning curve to achieve more minimally invasive interventions and less postoperative complications.

  6. Estudo comparativo entre os diagnósticos mielográfico anátomo-cirúrgico nas afecções raquemedulares

    Directory of Open Access Journals (Sweden)

    Celso Pereira da Silva

    1959-12-01

    Full Text Available O presente trabalho visa apreciar a exatidão das imagens mielográficas consideradas típicas pela maioria dos autores. Para êste fim os mielogramas de 161 casos operados foram divididos em 9 grupos, dos quais 7 (no total de 137 casos com imagens consideradas típicas e 2 (contendo 24 casos com imagens atípicas ou não conclusivas. Os diagnósticos mielográficos foram, a seguir, confrontados com os achados anátomo-cirúrgicos, obtendo-se uma concordância de 75% para o grupo dos angiomas, de 67,4% para o de hérnia de núcleo pulposo, de 59,2% para o de tumor extradural, de 45,4% para o de tumor intradural, de 33,3% para o de tumor intramedular, de 32,2% para o de aracnoidite e de 22,2% para o de hipertrofia do ligamento amarelo. A parte discordante em cada grupo abrangeu diagnósticos anátomo-cirúrgicos os mais variados. A análise estatística mostrou significância apenas para os grupos de tumor intramedular e hipertrofia do ligamento amarelo. A conclusão é a de que a imagem mielográfica pode contribuir para o diagnóstico da natureza das lesões obstrutivas dos espaços subaracnóideos; a sua exatidão porém não é absoluta, mas apenas relativa, sendo variável para cada entidade. A denominação habitualmente usada de "imagem típica" deve, por isso, ser substituída pela de "imagem sugestiva" de um determinado processo lesional.

  7. Treatment of Ruptured Vertebral Artery Dissecting Aneurysms

    Science.gov (United States)

    Hamasaki, Osamu; Ikawa, Fusao; Hidaka, Toshikazu; Kurokawa, Yasuharu; Yonezawa, Ushio

    2014-01-01

    Summary We evaluated the outcomes of endovascular or surgical treatment of ruptured vertebral artery dissecting aneurysms (VADAs), and investigated the relations between treatment complications and the development and location of the posterior inferior cerebellar artery (PICA). We treated 14 patients (12 men, two women; mean age, 56.2 years) with ruptured VADAs between March 1999 and June 2012 at our hospital. Six and eight patients had Hunt and Hess grades 1-3 and 4-5, respectively. Twelve patients underwent internal endovascular trapping, one underwent proximal endovascular occlusion alone, and one underwent proximal endovascular occlusion in the acute stage and occipital artery (OA)-PICA anastomosis and surgical trapping in the chronic stage. The types of VADA based on their location relative to the ipsilateral PICA were distal, PICA-involved, and non-PICA in nine, two, and three patients, respectively. The types of PICA based on their development and location were bilateral anterior inferior cerebellar artery (AICA)-PICA, ipsilateral AICA-PICA, extradural, and intradural type in one, two, two, and nine patients, respectively. Two patients with high anatomical risk developed medullary infarction, but their midterm outcomes were better than in previous reports. The modified Rankin scale indicated grades 0-2, 3-5, and 6 in eight, three, and three patients, respectively. A good outcome is often obtained in the treatment of ruptured VADA using internal endovascular trapping, except in the PICA-involved type, even with high-grade subarachnoid hemorrhage. Treatment of the PICA-involved type is controversial. The anatomical location and development of PICA may be predicted by complications with postoperative medullary infarction. PMID:24976093

  8. Trans-zygomatic middle cranial fossa approach to access lesions around the cavernous sinus and anterior parahippocampus: a minimally invasive skull base approach.

    Science.gov (United States)

    Melamed, Itay; Tubbs, R Shane; Payner, Troy D; Cohen-Gadol, Aaron A

    2009-08-01

    Exposure of the cavernous sinus or anterior parahippocampus often involves a wide exposure of the temporal lobe and mobilization of the temporalis muscle associated with temporal lobe retraction. The authors present a cadaveric study to illustrate the feasibility, advantages and landmarks necessary to perform a trans-zygomatic middle fossa approach to lesions around the cavernous sinus and anterior parahippocampus. The authors performed bilateral trans-zygomatic middle fossae exposures to reach the cavernous sinus and parahippocampus in five cadavers (10 sides). We assessed the morbidity associated with this procedure and compared the indications, advantages, and disadvantages of this method versus more extensive skull base approaches. A vertical linear incision along the middle portion of the zygomatic arch was extended one finger breadth inferior to the inferior edge of the zygomatic arch. Careful dissection inferior to the arch allowed preservation of facial nerve branches. A zygomatic osteotomy was followed via a linear incision through the temporalis muscle and exposure of the middle cranial fossa floor. A craniotomy along the inferolateral temporal bone and middle fossa floor allowed extradural dissection along the middle fossa floor and exposure of the cavernous sinus including all three divisions of the trigeminal nerve. Intradural inspection demonstrated adequate exposure of the parahippocampus. Exposure of the latter required minimal or no retraction of the temporal lobe. The trans-zygomatic middle fossa approach is a simplified skull base exposure using a linear incision, which may avoid the invasivity of more extensive skull base approaches while providing an adequate corridor for resection of cavernous sinus and parahippocampus lesions. The advantages of this approach include its efficiency, ease, minimalism, preservation of the temporalis muscle, and minimal retraction of the temporal lobe.

  9. Detection of residual disc hernia material and confirmation of nerve root decompression at lumbar disc herniation surgery by intraoperative ultrasound.

    Science.gov (United States)

    Aoyama, Takeshi; Hida, Kazutoshi; Akino, Minoru; Yano, Shunsuke; Iwasaki, Yoshinobu

    2009-06-01

    The aim of lumbar disc herniation surgery is the removal of herniated disc material (HDM) and complete decompression of the nerve root. As some patients present with residual HDM, we examined the ability of intraoperative ultrasound (IOUS) to detect this material. Between February 2006 and June 2007, we used IOUS in 30 patients undergoing surgery for lumbar disc herniation. They were 17 men and 13 women; their ages ranged from 22 to 63 y (mean 44.0 y). The level surgically addressed was L3/4 in 1, L4/5 in 14 and L5/S1 in 15 patients; they were operated in the prone position. After placing a 3-4 cm midline skin incision, partial hemi-semilaminotomy was performed. HDM was removed through a bone window; a surgical microscope was used during the operation. After removal was judged as adequate, IOUS was performed; 17 patients also underwent IOUS before removal of the herniated disc. For the acquisition of IOUS images, we used LOGIQ 9 and 8c microconvex probes (GE Healthcare, Wauwatosa, WI, USA). The normal anatomical structures were well visualized. HDM was iso- to hyperechoic compared with normal nerve tissue. In three of 17 patients, the dural sac and nerve root could not be distinguished from HDM before removal, although in all 30, the decompressed dural sac, intradural cauda equina and nerve root were well visualized. We posit that the echogenicity of nerve tissue was raised due to compression, rendering it similar to that of the herniated disc. In two patients, IOUS detected residual disc material; the surgical procedure was resumed and sufficient removal was accomplished. IOUS monitoring is safe, convenient and inexpensive. It is also highly useful for the detection of residual HDM and the confirmation of adequate nerve root decompression.

  10. Endovascular treatment of unruptured aneurysms of cavernous and ophthalmic segment of internal carotid artery with flow diverter device Pipeline.

    Science.gov (United States)

    Jevsek, Marko; Mounayer, Charbel; Seruga, Tomaz

    2016-12-01

    Intra-arterial treatment of aneurysms by redirecting blood flow is a newer method. The redirection is based on a significantly more densely braided wire stent. The stent wall keeps the blood in the lumen of the stent and slows down the turbulent flow in the aneurysms. Stagnation of blood in the aneurysm sac leads to the formation of thrombus and subsequent exclusion of the aneurysm from the circulation. The aim of the study was to evaluate flow diverter device Pipeline for broad neck and giant aneurysm treatment. Fifteen patients with discovered aneurysm of the internal carotid artery were treated between November 2010 and February 2014. The majority of aneurysms of the internal carotid artery were located intradural at the ophthalmic part of the artery. The patients were treated using a flow diverter device Pipeline, which was placed over the aneurysm neck. Treatment success was assessed clinically and angiographically using O'Kelly Marotta scale. Control angiography immediately after the release of the stent showed stagnation of the blood flow in the aneurysm sac. In none of the patients procedural and periprocedural complications were observed. 6 months after the procedure, control CT or MR angiography showed in almost all cases exclusion of the aneurysm from the circulation and normal blood flow in the treated artery. Neurological status six months after the procedure was normal in all patients. Treatment of aneurysms with flow diverter Pipeline device is a safe and significantly less time consuming method in comparison with standard techniques. This new method is a promising approach in treatment of broad neck aneurysms.

  11. MRI Findings of Early-Stage Hyperacute Hemorrhage Causing Extramedullary Compression of the Cervical Spinal Cord in a Dog with Suspected Steroid-Responsive Meningitis-Arteritis

    Directory of Open Access Journals (Sweden)

    Adriano Wang-Leandro

    2017-09-01

    Full Text Available A 9-month-old female Weimaraner was presented to the emergency service due to episodes of fever and neck pain. Physical examination revealed a stiff neck posture and elevated body temperature. Shortly after clinical examination was performed, the dog developed peracute onset of non-ambulatory tetraparesis compatible with a C1–C5 spinal cord (SC lesion. Immediately thereafter (<1 h, MRI of the cervical SC was performed with a 3-T scanner. A left ventrolateral intradural-extramedullary SC compression caused by a round-shaped structure at the level of C3––C4 was evidenced. The structure was iso- to slightly hyperintense in T1-weighted (T1W sequences compared to SC parenchyma and hyperintense in T2-weighted, gradient echo, and fluid-attenuated inversion recovery. Moreover, the structure showed a strong homogeneous contrast uptake in T1W sequences. Cerebrospinal fluid (CSF analysis revealed a mixed pleocytosis, as well as elevated protein and erythrocyte count. Early-stage hyperacute extramedullary hemorrhage was suspected due to immune mediated vasculitis. The dog was maintained under general anesthesia and artificial ventilation for 24 h and long-term therapy with corticosteroids and physiotherapy was initiated. Eight weeks after initial presentation, the dog was ambulatory, slightly tetraparetic. Follow-up MRI showed a regression of the round-shaped structure and pleocytosis was not evident in CSF analysis. This report describes an early-stage hyperacute extramedullary hemorrhage, a condition rarely recorded in dogs even in experimental settings.

  12. Integrated beam orientation and scanning-spot optimization in intensity-modulated proton therapy for brain and unilateral head and neck tumors.

    Science.gov (United States)

    Gu, Wenbo; O'Connor, Daniel; Nguyen, Dan; Yu, Victoria Y; Ruan, Dan; Dong, Lei; Sheng, Ke

    2018-02-02

    Intensity-Modulated Proton Therapy (IMPT) is the state-of-the-art method of delivering proton radiotherapy. Previous research has been mainly focused on optimization of scanning spots with manually selected beam angles. Due to the computational complexity, the potential benefit of simultaneously optimizing beam orientations and spot pattern could not be realized. In this study, we developed a novel integrated beam orientation optimization (BOO) and scanning-spot optimization algorithm for intensity-modulated proton therapy (IMPT). A brain chordoma and three unilateral head-and-neck patients with a maximal target size of 112.49 cm 3 were included in this study. A total number of 1162 noncoplanar candidate beams evenly distributed across 4π steradians were included in the optimization. For each candidate beam, the pencil-beam doses of all scanning spots covering the PTV and a margin were calculated. The beam angle selection and spot intensity optimization problem was formulated to include three terms: a dose fidelity term to penalize the deviation of PTV and OAR doses from ideal dose distribution; an L1-norm sparsity term to reduce the number of active spots and improve delivery efficiency; a group sparsity term to control the number of active beams between 2 and 4. For the group sparsity term, convex L2,1-norm and nonconvex L2,1/2-norm were tested. For the dose fidelity term, both quadratic function and linearized equivalent uniform dose (LEUD) cost function were implemented. The optimization problem was solved using the Fast Iterative Shrinkage-Thresholding Algorithm (FISTA). The IMPT BOO method was tested on three head-and-neck patients and one skull base chordoma patient. The results were compared with IMPT plans created using column generation selected beams or manually selected beams. The L2,1-norm plan selected spatially aggregated beams, indicating potential degeneracy using this norm. L2,1/2-norm was able to select spatially separated beams and achieve

  13. 2-[F-18] fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET) in uncommon malignancies

    International Nuclear Information System (INIS)

    Nair, N.; Basu, S.

    2004-01-01

    This is a retrospective study with an aim to evaluate the clinical role of FDG-PET imaging in changing the decision making process or management strategies in patients with various uncommon malignancies or in malignancies where there is paucity of literature regarding application of PET at present. The study population consisted of a wide variety of various uncommon malignancies who were mainly referred from the neighboring Tata Memorial Hospital with few cases from the outside centers. Patients were fasting at least for 6 hours. Sixty minutes after injection of 370 MBq FDG, patients were imaged on the dedicated BGO based GE Advance PET scanner (General Electric Medical systems, Milwaukee, WI). Images were reconstructed using the attenuation weighted Ordered Subsets Expectation Maximization (OSEM) algorithm. Axial, coronal, sagittal and 3D images were visually interpreted and foci of increased tracer uptake were considered as disease involvement. The findings were compared lesion by lesion with other imaging procedures regarding staging, treatment response evaluation and residual disease evaluation. The malignancies selected for retrospective analysis, along with the number of cases are presented. 2 cases of chordoma were evaluated of which one had primary in the cervicodorsal region and came for PET evaluation post surgery and RT. PET showed metastatic foci in left axillary node, body of sacrum and right lower neck region in addition to the persistence of disease in the primary. The other was a case of petroclival chordoma came for disease evaluation post surgery. The MRI was inconclusive regarding persistence of residual disease and postoperative changes. PET scan was normal in the case. In a solitary case of operated Dermato-fibrosarcoma, PET revealed a hypermetabolic focus at one end of scar, subsequently proven as scar recurrence of the primary. 3 cases of skin adenexal tumour were evaluated, all of whom were upstaged by PET and changed the subsequent

  14. Investigation of the added value of high-energy electrons in intensity-modulated radiotherapy: four clinical cases

    International Nuclear Information System (INIS)

    Korevaar, Erik W.; Huizenga, Henk; Loef, Johan; Stroom, Joep C.; Leer, Jan Willem H.; Brahme, Anders

    2002-01-01

    Purpose: Intensity-modulated radiotherapy (IMRT) with photon beams is currently pursued in many clinics. Theoretically, inclusion of intensity- and energy-modulated high-energy electron beams (15-50 MeV) offers additional possibilities to improve radiotherapy treatments of deep-seated tumors. In this study the added value of high-energy electron beams in IMRT treatments was investigated. Methods and Materials: In a comparative treatment planning study, conventional treatment plans and various types of IMRT plans were constructed for four clinical cases (cancer of the bladder, pancreas, chordoma of the sacrum, and breast). The conventional plans were used for the actual treatment of the patients. The IMRT plans were optimized using the Orbit optimization code (Loef et al., 2000) with a radiobiologic objective function. The IMRT plans were either photon or combined electron and photon beam plans, with or without dose homogeneity constraints assuming standard or increased radiosensitivities of organs at risk. Results: Large improvements in expected treatment outcome are found using IMRT plans compared to conventional plans, but differences in tumor control probability (TCP) and normal tissue complication probabilities (NTCP) values between IMRT plans with and without electrons are small. However, the use of electrons improves the dose-volume histograms for organs at risk, especially at lower dose levels (e.g., 0-40 Gy). Conclusions: This preliminary study indicates that addition of higher energy electrons to IMRT can only marginally improve treatment outcome for the selected cases. The dose-volume histograms of organs at risk show improvements for IMRT with higher energy electrons, which may reduce tumor induction but does not substantially reduce NTCP

  15. Typical tumors of the petrous bone; Typische Tumoren des Felsenbeins

    Energy Technology Data Exchange (ETDEWEB)

    Ahlhelm, F.; Mueller, U. [Kantonsspital Baden AG, Abteilung fuer Neuroradiologie, Institut fuer Radiologie, Baden (Switzerland); Ulmer, S. [Medizinisch-Radiologisches Institut, Zuerich (Switzerland)

    2014-04-15

    In the region of the petrous bone, inner acoustic canal and cerebellopontine angle, a variety of different tissues can be found, such as bony, epithelial, neural and vascular structures. Tumorous or tumor-like lesions, vascular or bony malformations or other pathologies can therefore be found in all of these areas. We discuss various frequently occurring tumorous or tumor-like pathologies including congential lesions, such as mucoceles, inflammatory disorders including osteomyelitis, pseudotumors and Wegener's granulomatosis. Benign non-neoplastic lesions, such as cholesteatoma, cholesterol granuloma, epidermoid and benign neoplastic tumors, such as the most commonly found vestibular schwannoma, meningeoma, paraganglioma, vascular pathologies and finally malignant lesions, such as metastasis, chordoma or chondrosarcoma and endolymphatic sac tumor (ELST) are also discussed. The emphasis of this article is on the appearance of these entities in computed tomography (CT) and more so magnetic resonance imaging (MRI), it provides key facts and typical images and discusses possibilities how to distinguish these pathologies. (orig.) [German] In der Region des Felsenbein, inneren Gehoerkanals und Kleinhirnbrueckenwinkels findet sich eine Vielzahl an unterschiedlichen Gewebearten inklusive knoechernes, epitheliales, nervales und vaskulaeres Gewebe. Tumoren oder tumoraehnliche Laesionen, ossaere oder vaskulaere Pathologien koennen entsprechend dort gefunden werden. Wir diskutieren verschiedene Tumoren oder tumoraehnliche Pathologien inklusive angeborene Laesionen wie Muko- und Meningozelen, entzuendliche Veraenderungen wie die Osteomyelitis, Pseudotumoren, die Wegener-Granulomatose, nichtneoplastische Tumoren wie das Epidermoid, Cholesteatom oder Cholesterolgranulom und gutartige neoplastische Tumoren wie das am haeufigsten zu findende Vestibularisschwannom, das Paragangliom und das Meningeom, Gefaessprozesse/-pathologien und schliesslich maligne Laesionen wie Metastasen

  16. Fine needle aspiration cytology in lesions of the nose, nasal cavity and paranasal sinuses.

    Science.gov (United States)

    Gupta, Nalini; Kaur, Jasleen; Srinivasan, Radhika; Das, Ashim; Mohindra, Satyawati; Rajwanshi, Arvind; Nijhawan, Raje

    2011-01-01

    To assess the spectrum of lesions in the sinonasal region diagnosed on FNAC. This is a retrospective audit of sinonasal lesions diagnosed on FNAC over a period of 12 years (1998-2009). Out of a total of 79,851 FNACs, 158 (0.2%) were from the sinonasal region. FNAC was non-diagnostic in 20 (12.6%) cases. Infective/inflammatory lesions comprised of 30 (19%) cases including non-specific inflammation (19), fungal infection (7), tuberculosis (2), actinomycosis (1) and filariasis (1). Benign cysts (24; 15.2%) included epidermal inclusion cysts, mucocele and aneurysmal bone cyst. Benign bone tumors (4) comprised of giant cell tumor, fibrous dysplasia, chondroma, and osteoblastoma. Other benign tumors included lipoma (6), hemangioma (5), schwannoma (2), meningioma (1), pleomorphic adenoma (1), sebaceous adenoma (1) and other skin adnexal tumors (3). Malignant epithelial tumors (24; 15.2%) included squamous cell carcinoma (10), basal cell carcinoma (5), poorly differentiated carcinoma (4) and metastatic carcinoma (5). Two cases of chordoma and one case each of dermatofibrosarcoma pertuberance and hemangiopericytoma were seen. Sarcomas included sarcoma, not otherwise specified (4), rhabdomyosarcoma (3), osteosarcoma (2), chondrosarcoma (2), leiomyosarcoma (1), malignant fibrous histiocytoma (1), fibrosarcoma (1) and malignant peripheral nerve sheath tumor (1). There were cases of malignant small round cell tumor (11), non-Hodgkin lymphoma (3), plasmacytoma (2) and malignant melanoma (2). A variety of non-neoplastic and neoplastic conditions can involve the sinonasal region. FNAC is a reliable diagnostic procedure in a good number of cases, especially in the light of clinico-radiological data. Copyright © 2011 S. Karger AG, Basel.

  17. Morphometric Analysis of Bone Resection in Anterior Petrosectomies.

    Science.gov (United States)

    Ahmed, Osama; Walther, Jonathan; Theriot, Krystle; Manuel, Morganne; Guthikonda, Bharat

    2016-06-01

    Introduction The anterior petrosectomy is a well-defined skull base approach to lesions such as petroclival meningiomas, posterior circulation aneurysms, petrous apex lesions (chondrosarcomas, cholesteatomas), ventrolateral brainstem lesions, clival chordomas, trigeminal neurinomas, and access to cranial nerves III, IV, V, and VII. Methods and Materials Fourteen anterior petrosectomies on eight cadaveric heads were performed in a skull base dissection laboratory. Predissection and postdissection thin-cut computed tomography scans were obtained to compare the bone resection. A computer program was used (InVivo5, Anatomage, San Jose, California, United States) to measure the bone resection and the improved viewing angle. Results The average bone removed in each plane was as follows: anterior to posterior plane was 10.57 mm ± 2.00 mm, superior to inferior was 9.39 mm ± 1.67 mm, and lateral to medial was 17.46 mm ± 4.64 mm. The average increased angle of view was 13.01 ± 2.35 degrees (Table 1). The average volume was 1786.94 ± 827.40 mm(3). Conclusions Anterior petrosectomy is a useful approach to access the ventrolateral brainstem region. We present a cadaveric study quantitating the volume of bone resection and improvement in the viewing angle. These data provide useful preoperative information on the utility of this skull base approach and the gain in the viewing angle after bony removal.

  18. Assessment of Early Toxicity and Response in Patients Treated With Proton and Carbon Ion Therapy at the Heidelberg Ion Therapy Center Using the Raster Scanning Technique

    International Nuclear Information System (INIS)

    Rieken, Stefan; Habermehl, Daniel; Nikoghosyan, Anna; Jensen, Alexandra; Haberer, Thomas; Jäkel, Oliver; Münter, Marc W.; Welzel, Thomas; Debus, Jürgen; Combs, Stephanie E.

    2011-01-01

    Puropose: To asses early toxicity and response in 118 patients treated with scanned ion beams to validate the safety of intensity-controlled raster scanning at the Heidelberg Ion Therapy Center. Patients and Methods: Between November 2009 and June 2010, we treated 118 patients with proton and carbon ion radiotherapy (RT) using active beam delivery. The main indications included skull base chordomas and chondrosarcomas, salivary gland tumors, and gliomas. We evaluated early toxicity within 6 weeks after RT and the initial clinical and radiologic response for quality assurance in our new facility. Results: In all 118 patients, few side effects were observed, in particular, no high numbers of severe acute toxicity were found. In general, the patients treated with particle therapy alone showed only a few single side effects, mainly Radiation Therapy Oncology Group/Common Terminology Criteria grade 1. The most frequent side effects and cumulative incidence of single side effects were observed in the head-and-neck patients treated with particle therapy as a boost and photon intensity-modulated RT. The toxicities included common radiation-attributed reactions known from photon RT, including mucositis, dysphagia, and skin erythema. The most predominant imaging responses were observed in patients with high-grade gliomas and those with salivary gland tumors. For skull base tumors, imaging showed a stable tumor outline in most patients. Thirteen patients showed improvement of pre-existing clinical symptoms. Conclusions: Side effects related to particle treatment were rare, and the overall tolerability of the treatment was shown. The initial response was promising. The data have confirmed the safe delivery of carbon ions and protons at the newly opened Heidelberg facility.

  19. Assessment of Early Toxicity and Response in Patients Treated With Proton and Carbon Ion Therapy at the Heidelberg Ion Therapy Center Using the Raster Scanning Technique

    Energy Technology Data Exchange (ETDEWEB)

    Rieken, Stefan; Habermehl, Daniel; Nikoghosyan, Anna; Jensen, Alexandra [Department of Radiation Oncology, University Hospital of Heidelberg, Heidelberg (Germany); Haberer, Thomas [Heidelberg Ion Therapy Center, Heidelberg (Germany); Jaekel, Oliver [Heidelberg Ion Therapy Center, Heidelberg (Germany); Department of Medical Physics, German Cancer Research Center (DKFZ), Heidelberg (Germany); Muenter, Marc W.; Welzel, Thomas; Debus, Juergen [Department of Radiation Oncology, University Hospital of Heidelberg, Heidelberg (Germany); Combs, Stephanie E., E-mail: Stephanie.Combs@med.uni-hedielberg.de [Department of Radiation Oncology, University Hospital of Heidelberg, Heidelberg (Germany)

    2011-12-01

    Puropose: To asses early toxicity and response in 118 patients treated with scanned ion beams to validate the safety of intensity-controlled raster scanning at the Heidelberg Ion Therapy Center. Patients and Methods: Between November 2009 and June 2010, we treated 118 patients with proton and carbon ion radiotherapy (RT) using active beam delivery. The main indications included skull base chordomas and chondrosarcomas, salivary gland tumors, and gliomas. We evaluated early toxicity within 6 weeks after RT and the initial clinical and radiologic response for quality assurance in our new facility. Results: In all 118 patients, few side effects were observed, in particular, no high numbers of severe acute toxicity were found. In general, the patients treated with particle therapy alone showed only a few single side effects, mainly Radiation Therapy Oncology Group/Common Terminology Criteria grade 1. The most frequent side effects and cumulative incidence of single side effects were observed in the head-and-neck patients treated with particle therapy as a boost and photon intensity-modulated RT. The toxicities included common radiation-attributed reactions known from photon RT, including mucositis, dysphagia, and skin erythema. The most predominant imaging responses were observed in patients with high-grade gliomas and those with salivary gland tumors. For skull base tumors, imaging showed a stable tumor outline in most patients. Thirteen patients showed improvement of pre-existing clinical symptoms. Conclusions: Side effects related to particle treatment were rare, and the overall tolerability of the treatment was shown. The initial response was promising. The data have confirmed the safe delivery of carbon ions and protons at the newly opened Heidelberg facility.

  20. One-step reconstruction with a 3D-printed, custom-made prosthesis after total en bloc sacrectomy: a technical note.

    Science.gov (United States)

    Wei, Ran; Guo, Wei; Ji, Tao; Zhang, Yidan; Liang, Haijie

    2017-07-01

    Surgeries for primary malignancies involving upper sacrum require total en bloc sacrectomy followed by complex mechanical reconstruction, which might be simplified by application of the three-dimensional (3D) printing technique. To describe the design of a 3D-printed custom-made prosthesis for reconstruction after total en bloc sacrectomy, the surgical technique, and the clinical and functional outcome of a patient. A 62-year-old patient with recurrent sacral chordoma was admitted in our center. One-stage total en bloc sacrectomy through posterior approach was planned, and a 3D-printed sacral prosthesis was prepared for reconstruction according to the anticipated osteotomic planes. The patient received one-stage total en bloc sacrectomy through posterior approach followed by reconstruction with the 3D-printed sacral prosthesis. The whole procedure took 5 h, and intra-operative blood loss was 3400 ml. The patient recovered uneventfully and started ambulation at 3 weeks after surgery. An asymptomatic instrument failure was found radiographically at 8-month follow-up. At 1 year after surgery, the patient was disease free and could walk over short distance with crutches without pain or any mechanical instability. The advantages of our reconstruction method included: (1) the prosthesis provided an optimal reconstruction of lumbosacral and pelvic ring by integrating spinal pelvic fixation, posterior pelvic ring fixation, and anterior spinal column fixation in one step and (2) its porous surface could induce bone ingrowth and might enhance stability. Although there was an instrumental failure, we considered that it could be one reconstructive option. More research is warranted focusing on the modification of locations, diameters, and quantity of screws and biomechanical characteristics. The long-term functional and bone in-growth outcome will be followed to validate the use of the prosthesis.

  1. Risk factors for postoperative cerebrospinal fluid leak and meningitis after expanded endoscopic endonasal surgery.

    Science.gov (United States)

    Ivan, Michael E; Iorgulescu, J Bryan; El-Sayed, Ivan; McDermott, Michael W; Parsa, Andrew T; Pletcher, Steven D; Jahangiri, Arman; Wagner, Jeffrey; Aghi, Manish K

    2015-01-01

    Postoperative cerebrospinal fluid (CSF) leak is a serious complication of transsphenoidal surgery, which can lead to meningitis and often requires reparative surgery. We sought to identify preoperative risk factors for CSF leaks and meningitis. We reviewed 98 consecutive expanded endoscopic endonasal surgeries performed from 2008-2012 and analyzed preoperative comorbidities, intraoperative techniques, and postoperative care. Univariate and multivariate analyses were performed. The most common pathologies addressed included pituitary adenoma, Rathke cyst, chordoma, esthesioneuroblastoma, meningioma, nasopharyngeal carcinoma, and squamous cell carcinoma. There were 11 CSF leaks (11%) and 10 central nervous system (CNS) infections (10%). Univariate and multivariate analysis of preoperative risk factors showed that patients with non-ideal body mass index (BMI) were associated with higher rate of postoperative CSF leak and meningitis (both p<0.01). Also, patients with increasing age were associated with increased CSF leak (p = 0.03) and the length of time a lumbar drain was used postoperatively was associated with infection in a univariate analysis. In addition, three of three endoscopic transsphenoidal surgeries combined with open cranial surgery had a postoperative CSF leak and CNS infection rate which was a considerably higher rate than for transsphenoidal surgeries alone or surgeries staged with open cases (p<0.01 and p=0.04, respectively) In this series of expanded endoscopic transsphenoidal surgeries, preoperative BMI remains the most important preoperative predictor for CSF leak and infection. Other risk factors include age, intraoperative CSF leak, lumbar drain duration, and cranial combined cases. Risks associated with complex surgical resections when combining open and endoscopic approaches could be minimized by staging these procedures. Copyright © 2014 Elsevier Ltd. All rights reserved.

  2. Perioperative fractionated high-dose rate brachytherapy for malignant bone and soft tissue tumors

    International Nuclear Information System (INIS)

    Koizumi, Masahiko; Inoue, Takehiro; Yamazaki, Hideya; Teshima, Teruki; Tanaka, Eiichi; Yoshida, Ken; Imai, Atsushi; Shiomi, Hiroya; Kagawa, Kazufumi; Araki, Nobuto; Kuratsu, Shigeyuki; Uchida, Atsumasa; Inoue, Toshihiko

    1999-01-01

    Purpose: To investigate the viability of perioperative fractionated HDR brachytherapy for malignant bone and soft tissue tumors, analyzing the influence of surgical margin. Methods and Materials: From July 1992 through May 1996, 16 lesions of 14 patients with malignant bone and soft tissue tumors (3 liposarcomas, 3 MFHs, 2 malignant schwannomas, 2 chordomas, 1 osteosarcoma, 1 leiomyosarcoma, 1 epithelioid sarcoma, and 1 synovial sarcoma) were treated at the Osaka University Hospital. The patients' ages ranged from 14 to 72 years (median: 39 years). Treatment sites were the pelvis in 6 lesions, the upper limbs in 5, the neck in 4, and a lower limb in 1. The resection margins were classified as intracapsular in 5 lesions, marginal in 5, and wide in 6. Postoperative fractionated HDR brachytherapy was started on the 4th-13th day after surgery (median: 6th day). The total dose was 40-50 Gy/7-10 fr/ 4-7 day (bid) at 5 or 10 mm from the source. Follow-up periods were between 19 and 46 months (median: 30 months). Results: Local control rates were 75% at 1 year and 48% in 2 years, and ultimate local control was achieved in 8 (50%) of 16 lesions. Of the 8 uncontrolled lesions, 5 (63%) had intracapsular (macroscopically positive) resection margins, and all the 8 controlled lesions (100%) had marginal (microscopically positive) or wide (negative) margins. Of the total, 3 patients died of both tumor and metastasis, 3 of metastasis alone, 1 of tumor alone, and 7 showed no evidence of disease. Peripheral nerve palsy was seen in one case after this procedure, but no infection or delayed wound healing caused by tubing or irradiation has occurred. Conclusion: Perioperative fractionated HDR brachytherapy is safe, well tolerated, and applicable to marginal or wide surgical margin cases

  3. Endoscopic endonasal transsphenoidal surgery using the iArmS operation support robot: initial experience in 43 patients.

    Science.gov (United States)

    Ogiwara, Toshihiro; Goto, Tetsuya; Nagm, Alhusain; Hongo, Kazuhiro

    2017-05-01

    Objective The intelligent arm-support system, iArmS, which follows the surgeon's arm and automatically fixes it at an adequate position, was developed as an operation support robot. iArmS was designed to support the surgeon's forearm to prevent hand trembling and to alleviate fatigue during surgery with a microscope. In this study, the authors report on application of this robotic device to endoscopic endonasal transsphenoidal surgery (ETSS) and evaluate their initial experiences. Methods The study population consisted of 43 patients: 29 with pituitary adenoma, 3 with meningioma, 3 with Rathke's cleft cyst, 2 with craniopharyngioma, 2 with chordoma, and 4 with other conditions. All patients underwent surgery via the endonasal transsphenoidal approach using a rigid endoscope. During the nasal and sphenoid phases, iArmS was used to support the surgeon's nondominant arm, which held the endoscope. The details of the iArmS and clinical results were collected. Results iArmS followed the surgeon's arm movement automatically. It reduced the surgeon's fatigue and stabilized the surgeon's hand during ETSS. Shaking of the video image decreased due to the steadying of the surgeon's scope-holding hand with iArmS. There were no complications related to use of the device. Conclusions The intelligent armrest, iArmS, seems to be safe and effective during ETSS. iArmS is helpful for improving the precision and safety not only for microscopic neurosurgery, but also for ETSS. Ongoing advances in robotics ensure the continued evolution of neurosurgery.

  4. A panoramic view of the skull base: systematic review of open and endoscopic endonasal approaches to four tumors.

    Science.gov (United States)

    Graffeo, Christopher S; Dietrich, August R; Grobelny, Bartosz; Zhang, Meng; Goldberg, Judith D; Golfinos, John G; Lebowitz, Richard; Kleinberg, David; Placantonakis, Dimitris G

    2014-08-01

    Endoscopic endonasal surgery has been established as the safest approach to pituitary tumors, yet its role in other common skull base lesions has not been established. To answer this question, we carried out a systematic review of reported series of open and endoscopic endonasal approaches to four major skull base tumors: olfactory groove meningiomas (OGM), tuberculum sellae meningiomas (TSM), craniopharyngiomas (CRA), and clival chordomas (CHO). Data from 162 studies containing 5,701 patients were combined and compared for differences in perioperative mortality, gross total resection (GTR), cerebrospinal fluid (CSF) leak, neurological morbidity, post-operative visual function, post-operative anosmia, post-operative diabetes insipidus (DI), and post-operative obesity/hyperphagia. Weighted average rates for each outcome were calculated using relative study size. Our findings indicate similar rates of GTR and perioperative mortality between open and endoscopic approaches for all tumor types. CSF leak was increased after endoscopic surgery. Visual function symptoms were more likely to improve after endoscopic surgery for TSM, CRA, and CHO. Post-operative DI and obesity/hyperphagia were significantly increased after open resection in CRA. Recurrence rates per 1,000 patient-years of follow-up were higher in endoscopy for OGM, TSM, and CHO. Trends for open and endoscopic surgery suggested modest improvement in all outcomes over time. Our observations suggest that endonasal endoscopy is a safe alternative to craniotomy and may be preferred for certain tumor types. However, endoscopic surgery is associated with higher rates of CSF leak, and possibly increased recurrence rates. Prospective study with long-term follow-up is required to verify these preliminary observations.

  5. SU-F-T-214: Re-Thinking the Useful Clinical Beam Energy in Proton Therapy: An Opportunity for Cost Reduction

    Energy Technology Data Exchange (ETDEWEB)

    Bentefour, El H [IBA, Advanced Technology Group, Louvain La Neuve (Belgium); Lu, H [Massachusetts General Hospital and Harvard Medical School, Boston, MA (United States)

    2016-06-15

    Purpose: We conducted a retrospective study of the useful clinical proton beam energy based on the beam range data of patients treated over the last 10 years at Massachusetts General Hospital Proton Therapy Center. Methods: Treatment field information were collected for all patients treated over the last 10 years (2005–2015) in the two gantry treatment rooms at MGH. The beam ranges for these fields were retrieved and categorized per treatment site. The 10 prostate patients that required the highest beam range (lateral fields) were selected. For these patients, anterior oblique beams (30–40 degrees) were simulated in a planning system to obtain the required beam ranges including the margins for potential range uncertainties. Results: There were a total of 4033 patients, treated with combined total of 23603 fields. All treatment indications were considered with the exception of ocular tumors generally treated in a fixed beam room. For all non-prostate treatments (21811 fields), only 5 fields for 4 patients (1-pancreas, 1-lumbar chordoma, 2-spine mets) required beam range greater than 25 cm. There were 446 prostate patients (1792 fields), with the required beam range from 22.3 to 29.0 cm; 386 of them had at least one of their lateral beam range greater than 25 cm. For the 10 prostate patients with highest lateral beam ranges (26 to 29 cm), their treatment with anterior oblique beams would drop the beam ranges below 25 cm (17.3 to 18.5 cm). Conclusion: if prostate patients are treated with anterior fields only, the useful maximum beam range is reduced to 25 cm. Thus a proton therapy system with maximum beam energy of 196 MeV is sufficient to treat all tumors sites with very rare exceptions (<0.1%). Designing such PT system would reduce the cost of proton therapy for hospitals and patients and increase the accessibility to the treatment.

  6. Reconstruction of extensive defects from posterior en bloc resection of sacral tumors with human acellular dermal matrix and gluteus maximus myocutaneous flaps.

    Science.gov (United States)

    Dasenbrock, Hormuzdiyar H; Clarke, Michelle J; Bydon, Ali; Witham, Timothy F; Sciubba, Daniel M; Simmons, Oliver P; Gokaslan, Ziya L; Wolinsky, Jean-Paul

    2011-12-01

    Performing a sacrectomy from an exclusively posterior approach allows the en bloc resection of tumors without the morbidity of a laparotomy. However, reconstruction of the resultant extensive soft-tissue defects is challenging because a vertical rectus abdominis myocutaneous flap is not harvested. To report the largest series (with the longest follow-up) of sacral reconstructions using a combination of human acellular dermal matrix (HADM) and gluteus maximus myocutaneous flaps. Thirty-four patients with sacral tumors with a follow-up of at least 1 year were reviewed retrospectively. After the tumor was excised, HADM (AlloDerm, LifeCell Corp, Branchburg, New Jersey) was secured to create a pelvic diaphragm. Subsequently, the gluteus maximus muscles were freed from their origins and advanced to cover the HADM. The mean age of patients was 50.1 years (SD, 16.0 years), and the histopathology was a chordoma in 82.4%. Seven patients (20.6%) developed a postoperative wound dehiscence, 5 of whom (14.7%) required operative debridement. An estimated blood loss of >1500 mL, an operative time of >9 hours during sacrectomy, and postoperative bowel incontinence were associated with a significantly higher likelihood of undergoing a subsequent debridement for dehiscence (P ≤ .03). With a mean follow-up of 45.7 months, only 1 patient developed an asymptomatic parasacral hernia. Reconstruction of posterior sacrectomy defects with HADM and gluteus maximus myocutaneous flaps may be valid. This approach may have rates of wound dehiscence comparable to other techniques and low rates of parasacral herniation.

  7. Proton Therapy in Children: A Systematic Review of Clinical Effectiveness in 15 Pediatric Cancers

    Energy Technology Data Exchange (ETDEWEB)

    Leroy, Roos, E-mail: Roos.leroy@kce.fgov.be [Belgian Healthcare Knowledge Centre (KCE), Brussels (Belgium); Benahmed, Nadia; Hulstaert, Frank [Belgian Healthcare Knowledge Centre (KCE), Brussels (Belgium); Van Damme, Nancy [Belgian Cancer Registry, Brussels (Belgium); De Ruysscher, Dirk [Department of Radiation Oncology, University of Leuven, Leuven (Belgium)

    2016-05-01

    Because it spares many normal tissues and reduces the integral dose, proton therapy (PT) is the preferred tumor irradiation technique for treating childhood cancer. However, to the best of our knowledge, no systematic review of the clinical effectiveness of PT in children has been reported in the scientific literature. A systematic search for clinical outcome studies on PT published between 2007 and 2015 was performed in Medline (through OVID), EMBASE, and the Cochrane Library. Twenty-three primary studies were identified, including approximately 650 patients overall. The median/mean follow-up times were limited (range, 19-91 months). None of the studies were randomized, 2 were comparative, and 20 were retrospective. Most suffered from serious methodologic limitations, yielding a very low level of clinical evidence for the outcomes in all indications. For example, for retinoblastoma, very low-level evidence was found that PT might decrease the incidence of second malignancies. For chondrosarcoma, chordoma, craniopharyngioma, ependymoma, esthesioneuroblastoma, Ewing sarcoma, central nervous system germinoma, glioma, medulloblastoma, osteosarcoma, and rhabdomyosarcoma, there was insufficient evidence to either support or refute PT in children. For pelvic sarcoma (ie, nonrhabdomyosarcoma and non-Ewing sarcoma), pineal parenchymal tumor, primitive neuroectodermal tumor, and “adult-type” soft tissue sarcoma, no studies were identified that fulfilled the inclusion criteria. Although there is no doubt that PT reduces the radiation dose to normal tissues and organs, to date the critical clinical data on the long-term effectiveness and harm associated with the use of PT in the 15 pediatric cancers under investigation are lacking. High-quality clinical research in this area is needed.

  8. Reoptimization of Intensity Modulated Proton Therapy Plans Based on Linear Energy Transfer

    Energy Technology Data Exchange (ETDEWEB)

    Unkelbach, Jan, E-mail: junkelbach@mgh.harvard.edu [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Botas, Pablo [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Faculty of Physics, Ruprecht-Karls-Universität Heidelberg, Heidelberg (Germany); Giantsoudi, Drosoula; Gorissen, Bram L.; Paganetti, Harald [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States)

    2016-12-01

    Purpose: We describe a treatment plan optimization method for intensity modulated proton therapy (IMPT) that avoids high values of linear energy transfer (LET) in critical structures located within or near the target volume while limiting degradation of the best possible physical dose distribution. Methods and Materials: To allow fast optimization based on dose and LET, a GPU-based Monte Carlo code was extended to provide dose-averaged LET in addition to dose for all pencil beams. After optimizing an initial IMPT plan based on physical dose, a prioritized optimization scheme is used to modify the LET distribution while constraining the physical dose objectives to values close to the initial plan. The LET optimization step is performed based on objective functions evaluated for the product of LET and physical dose (LET×D). To first approximation, LET×D represents a measure of the additional biological dose that is caused by high LET. Results: The method is effective for treatments where serial critical structures with maximum dose constraints are located within or near the target. We report on 5 patients with intracranial tumors (high-grade meningiomas, base-of-skull chordomas, ependymomas) in whom the target volume overlaps with the brainstem and optic structures. In all cases, high LET×D in critical structures could be avoided while minimally compromising physical dose planning objectives. Conclusion: LET-based reoptimization of IMPT plans represents a pragmatic approach to bridge the gap between purely physical dose-based and relative biological effectiveness (RBE)-based planning. The method makes IMPT treatments safer by mitigating a potentially increased risk of side effects resulting from elevated RBE of proton beams near the end of range.

  9. Brainstem tolerance to conformal radiotherapy of skull base tumors

    International Nuclear Information System (INIS)

    Debus, J.; Hug, E.B.; Liebsch, N.J.; O'Farrel, D.; Finkelstein, D.; Efird, J.; Munzenrider, J.E.

    1997-01-01

    Purpose: The aim of this study was to analyze the long-term incidence of brainstem toxicity in patients treated for skull base tumors with high dose conformal radiotherapy. Methods and Materials: Between 1974 and 1995, 367 patients with chordomas (n = 195) and chondrosarcomas (n = 172) of the base of skull have been treated with combined megavoltage photon and 160 MeV proton radiotherapy. Following 3D treatment planning with delineation of target volumes and critical nontarget structures dose distributions and dose-volume histograms were calculated. Radiotherapy was given an 1.8 Gy or CGE (=Cobalt Gray Equivalent) dose per fraction, with prescribed target doses ranging from 63 CGE to 79.2 CGE (mean = 67.8 CGE). Doses to the brainstem surface were limited to ≤64 CGE and to the brainstem center to ≤53 CGE. Results: Follow-up time ranged from 6 months to 21.4 years (mean = 42.5 months). Brainstem toxicity was observed in 17 of 367 patients attributable to treatment, resulting in death of three patients. Actuarial rates of 5 and 10-year high-grade toxicity-free survival were 94 and 88%, respectively. Increased risk of brainstem toxicity was significantly associated with maximum dose to brainstem, volume of brainstem receiving ≥50 CGE, ≥55 CGE, and ≥60 CGE, number of surgical procedures, and prevalence of diabetes or high blood pressure. Multivariate analysis identified three independent factors as important prognosticators: number of surgical procedures (p < 0.001), volume of the brainstem receiving 60 CGE (p < 0.001), and prevalence of diabetes (p < 0.01). Conclusions: Tolerance of brainstem to fractionated radiotherapy appears to be a steep function of tissue volume included in high dose regions rather than the maximum dose of brainstem alone. In addition, presence of predisposing factors as well as extent of surgical manipulation can significantly lower brainstem tolerance in the individual patient

  10. Activating mutations in c-KIT and PDGFRalpha are exclusively found in gastrointestinal stromal tumors and not in other tumors overexpressing these imatinib mesylate target genes.

    Science.gov (United States)

    Burger, Herman; den Bakker, Michael A; Kros, Johan M; van Tol, Hans; de Bruin, Alex M; Oosterhuis, Wolter; van den Ingh, Harry F G M; van der Harst, Erwin; de Schipper, Hans P; Wiemer, Erik A C; Nooter, Kees

    2005-11-01

    Previous studies have shown that Imatinib mesylate (Gleevec), a selective tyrosine kinase inhibitor of c-KIT and platelet-derived growth factor receptors (PDGFR), is highly effective in c-KIT/CD117-positive gastrointestinal stromal tumors (GIST), especially in those having activating mutations in c-kit exon 11. In addition, gain-of-function mutations in the juxtamembrane domain (exon 12) and the kinase activation loop (exon 18) of PDGFRalpha were found in GISTs. Importantly, the presence and type of these mutually exclusive c-KIT or PDGFRalpha mutations were found to be associated with the response to imatinib. Here, we examined the prevalence of c-kit exon 11 and PDGFRalpha exons 12 and 18 mutations in other tumor types known to express these tyrosine kinase receptors in order to explore which other cancer types may potentially benefit from imatinib treatment. We determined the mutational status of these commonly mutated exons by direct sequencing in 11 different tumor types (in total: 215 unrelated cases), including GIST, chordoma, and various distinct tumors of lung, brain and its coverings, and skin cancer. Of the 579 exons examined (211 c-kit exon 11, 192 PDGFRalpha exon 12, 142 PDGFRalpha exon18, 17 PDGFRbeta exon 12 and 17 PDGFRbeta exon 18), only 12 (all GIST) harbored mutations (10 c-kit exon 11 and 2 PDGFRalpha exon18). From these data we conclude that activating c-KIT and PDGFR mutations are sporadic in human cancers known to overexpress these tyrosine kinase receptor genes and suggest that, except in GIST, this overexpression is not correlated with activating mutations. The latter may imply that these wild-type c-KIT and PDGFR tumor types will probably not benefit from imatinib treatment.

  11. Endocrine function following high dose proton therapy for tumors of the upper clivus

    Energy Technology Data Exchange (ETDEWEB)

    Slater, J.D.; Austin-Seymour, M.; Munzenrider, J.; Birnbaum, S.; Carroll, R.; Klibanski, A.; Riskind, P.; Urie, M.; Verhey, L.; Goitein, M.

    1988-09-01

    The endocrine status of patients receiving proton radiation for tumors of the upper clivus was reviewed to evaluate the effect of high dose treatment on the pituitary gland. The fourteen patients had chordomas or low grade chondrosarcomas and were all treated by the same techniques. The median tumor dose was 69.7 Cobalt Gray Equivalent (CGE) with a range from 66.6 to 74.4 CGE. (CGE is used because modulated protons have an RBE of 1.1 compared to 60Co). The daily fraction size was 1.8-2.1 CGE. The median follow-up time is 48 months, ranging from 30 to 68 months. All treatments were planned using a computerized multi-dimensional system with the position of the pituitary outlined on the planning CT scan. Review of the dose distribution indicated that the dose to the pituitary ranged from 60.5 to 72.3 CGE, with a median of 67.6 CGE. One female patient had decreased thyroid and gonadotropin function at the time of diagnosis and has been on hormone replacement since that time. The other three females were all pre-menopausal at the time of radiotherapy. At this time four patients (3 males and 1 female) have developed endocrine abnormalities 14 to 45 months after irradiation. All four had evidence of hypothyroidism and two have also developed corticotropin deficiency. The three males had decreased testosterone levels; the female patient developed amenorrhea and hyperprolactinemia. All four are asymptomatic with ongoing hormone replacement.

  12. Endocrine function following high dose proton therapy for tumors of the upper clivus

    International Nuclear Information System (INIS)

    Slater, J.D.; Austin-Seymour, M.; Munzenrider, J.

    1988-01-01

    The endocrine status of patients receiving proton radiation for tumors of the upper clivus was reviewed to evaluate the effect of high dose treatment on the pituitary gland. The fourteen patients had chordomas or low grade chondrosarcomas and were all treated by the same techniques. The median tumor dose was 69.7 Cobalt Gray Equivalent (CGE) with a range from 66.6 to 74.4 CGE. (CGE is used because modulated protons have an RBE of 1.1 compared to 60Co). The daily fraction size was 1.8-2.1 CGE. The median follow-up time is 48 months, ranging from 30 to 68 months. All treatments were planned using a computerized multi-dimensional system with the position of the pituitary outlined on the planning CT scan. Review of the dose distribution indicated that the dose to the pituitary ranged from 60.5 to 72.3 CGE, with a median of 67.6 CGE. One female patient had decreased thyroid and gonadotropin function at the time of diagnosis and has been on hormone replacement since that time. The other three females were all pre-menopausal at the time of radiotherapy. At this time four patients (3 males and 1 female) have developed endocrine abnormalities 14 to 45 months after irradiation. All four had evidence of hypothyroidism and two have also developed corticotropin deficiency. The three males had decreased testosterone levels; the female patient developed amenorrhea and hyperprolactinemia. All four are asymptomatic with ongoing hormone replacement

  13. Nivolumab and Ipilimumab in Treating Patients With Rare Tumors

    Science.gov (United States)

    2018-04-23

    Acinar Cell Carcinoma; Adenoid Cystic Carcinoma; Adrenal Cortex Carcinoma; Adrenal Gland Pheochromocytoma; Anal Canal Neuroendocrine Carcinoma; Anal Canal Undifferentiated Carcinoma; Appendix Mucinous Adenocarcinoma; Bartholin Gland Transitional Cell Carcinoma; Bladder Adenocarcinoma; Cervical Adenocarcinoma; Cholangiocarcinoma; Chordoma; Colorectal Squamous Cell Carcinoma; Desmoid-Type Fibromatosis; Endometrial Transitional Cell Carcinoma; Endometrioid Adenocarcinoma; Esophageal Neuroendocrine Carcinoma; Esophageal Undifferentiated Carcinoma; Extrahepatic Bile Duct Carcinoma; Fallopian Tube Adenocarcinoma; Fallopian Tube Transitional Cell Carcinoma; Fibromyxoid Tumor; Gastric Neuroendocrine Carcinoma; Gastric Squamous Cell Carcinoma; Gastrointestinal Stromal Tumor; Giant Cell Carcinoma; Intestinal Neuroendocrine Carcinoma; Intrahepatic Cholangiocarcinoma; Lung Carcinoid Tumor; Lung Sarcomatoid Carcinoma; Major Salivary Gland Carcinoma; Malignant Odontogenic Neoplasm; Malignant Peripheral Nerve Sheath Tumor; Malignant Testicular Sex Cord-Stromal Tumor; Metaplastic Breast Carcinoma; Metastatic Malignant Neoplasm of Unknown Primary Origin; Minimally Invasive Lung Adenocarcinoma; Mixed Mesodermal (Mullerian) Tumor; Mucinous Adenocarcinoma; Mucinous Cystadenocarcinoma; Nasal Cavity Adenocarcinoma; Nasal Cavity Carcinoma; Nasopharyngeal Carcinoma; Nasopharyngeal Papillary Adenocarcinoma; Nasopharyngeal Undifferentiated Carcinoma; Oral Cavity Carcinoma; Oropharyngeal Undifferentiated Carcinoma; Ovarian Adenocarcinoma; Ovarian Germ Cell Tumor; Ovarian Mucinous Adenocarcinoma; Ovarian Squamous Cell Carcinoma; Ovarian Transitional Cell Carcinoma; Pancreatic Acinar Cell Carcinoma; Pancreatic Neuroendocrine Carcinoma; Paraganglioma; Paranasal Sinus Adenocarcinoma; Paranasal Sinus Carcinoma; Parathyroid Gland Carcinoma; Pituitary Gland Carcinoma; Placental Choriocarcinoma; Placental-Site Gestational Trophoblastic Tumor; Primary Peritoneal High Grade Serous Adenocarcinoma

  14. A radiobiological Markov simulation tool for aiding decision making in proton therapy referral.

    Science.gov (United States)

    Austin, Annabelle M; Douglass, Michael J J; Nguyen, Giang T; Penfold, Scott N

    2017-12-01

    Proton therapy can be a highly effective strategy for the treatment of tumours. However, compared with X-ray therapy it is more expensive and has limited availability. In addition, it is not always clear whether it will benefit an individual patient more than a course of traditional X-ray therapy. Basing a treatment decision on outcomes of clinical trials can be difficult due to a shortage of data. Predictive modelling studies are becoming an attractive alternative to supplement clinical decisions. The aim of the current work is to present a Markov framework that compares clinical outcomes for proton and X-ray therapy. A Markov model has been developed which estimates the radiobiological effect of a given treatment plan. This radiobiological effect is estimated using the tumour control probability (TCP), normal tissue complication probability (NTCP) and second primary cancer induction probability (SPCIP). These metrics are used as transition probabilities in the Markov chain. The clinical outcome is quantified by the quality adjusted life expectancy. To demonstrate functionality, the model was applied to a 6-year-old patient presenting with skull base chordoma. The model was successfully developed to compare clinical outcomes for proton and X-ray treatment plans. For the example patient considered, it was predicted that proton therapy would offer a significant advantage compared with volumetric modulated arc therapy in terms of survival and mitigating injuries. The functionality of the model was demonstrated using the example patient. The proposed Markov method may be a useful tool for deciding on a treatment strategy for individual patients. Crown Copyright © 2017. Published by Elsevier Ltd. All rights reserved.

  15. Imaging appearances and clinical outcome following sacrectomy and ilio-lumbar reconstruction for sacral neoplasia

    Energy Technology Data Exchange (ETDEWEB)

    Thomas, Marianna; Davies, A.M.; James, Steven L.J. [Department of Radiology, The Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham (United Kingdom); Stirling, A.J.; Grainger, M. [Department of Spinal Surgery, The Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham (United Kingdom); Grimer, R.J. [Department of Orthopaedic Oncology, The Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham (United Kingdom)

    2014-02-15

    Sacrectomy and ilio-lumbar reconstruction is an uncommonly performed complex surgical procedure for the treatment of sacral neoplasia. There are many challenges in the post-operative period including the potential for tumor recurrence, infection, and construct failure. We present our experience of this patient cohort and describe the complications and imaging appearances that can be encountered during the follow-up period. Retrospective review of our Orthopaedic Oncology database was undertaken which has been collected over a 30-year period to identify patients that had undergone sacrectomy and ilio-lumbar reconstruction. Pre and post-operative imaging including radiographs, CT, and MRI was reviewed. These were viewed by two experienced musculoskeletal radiologists with consensus opinion if there was disagreement over the imaging findings. Data regarding patient demographics, tumor type, and dimensions was collected. Serial review of radiographs, CT, and MRI was performed to assess implant position and integrity, strut graft position and union, and for the presence of recurrence within the surgical bed. Five male and two female patients (mean age 36 years, age range 15-54 years) were treated with this procedure. Histological diagnoses included chordoma, chondrosarcoma, osteosarcoma, and spindle cell sarcoma. Mean maximal tumor size on pre-operative imaging was 10.7 cm (range, 6-16 cm). Post-operative follow-up ranged from 10-46 months. A total of 76 imaging studies were reviewed. Commonly identified complications included vertical rod and cross-connector fracture and screw loosening. Fibula strut graft non-union and fracture was also evident on imaging review. Two patients demonstrated disease recurrence during the follow-up period. This study demonstrates the spectrum and frequency of complications that can occur following sacrectomy and ilio-lumbar reconstruction for sacral neoplasia. (orig.)

  16. Training to acquire psychomotor skills for endoscopic endonasal surgery using a personal webcam trainer.

    Science.gov (United States)

    Hirayama, Ryuichi; Fujimoto, Yasunori; Umegaki, Masao; Kagawa, Naoki; Kinoshita, Manabu; Hashimoto, Naoya; Yoshimine, Toshiki

    2013-05-01

    Existing training methods for neuroendoscopic surgery have mainly emphasized the acquisition of anatomical knowledge and procedures for operating an endoscope and instruments. For laparoscopic surgery, various training systems have been developed to teach handling of an endoscope as well as the manipulation of instruments for speedy and precise endoscopic performance using both hands. In endoscopic endonasal surgery (EES), especially using a binostril approach to the skull base and intradural lesions, the learning of more meticulous manipulation of instruments is mandatory, and it may be necessary to develop another type of training method for acquiring psychomotor skills for EES. Authors of the present study developed an inexpensive, portable personal trainer using a webcam and objectively evaluated its utility. Twenty-five neurosurgeons volunteered for this study and were divided into 2 groups, a novice group (19 neurosurgeons) and an experienced group (6 neurosurgeons). Before and after the exercises of set tasks with a webcam box trainer, the basic endoscopic skills of each participant were objectively assessed using the virtual reality simulator (LapSim) while executing 2 virtual tasks: grasping and instrument navigation. Scores for the following 11 performance variables were recorded: instrument time, instrument misses, instrument path length, and instrument angular path (all of which were measured in both hands), as well as tissue damage, max damage, and finally overall score. Instrument time was indicated as movement speed; instrument path length and instrument angular path as movement efficiency; and instrument misses, tissue damage, and max damage as movement precision. In the novice group, movement speed and efficiency were significantly improved after the training. In the experienced group, significant improvement was not shown in the majority of virtual tasks. Before the training, significantly greater movement speed and efficiency were demonstrated in

  17. Surgical treatment of spinal ependymoma and post-operative radiotherapy

    International Nuclear Information System (INIS)

    Lee, T.T.; Gromelski, E.B.; Green, B.A.

    1998-01-01

    With the advances in microsurgical and monitoring techniques, spinal ependymomas are gross totally resected more frequently. The use of adjuvant radiotherapy has become questionable with gross total resection and its role for residual neoplasm need to be redefined. A retrospective analysis of a series of patients was carried out to investigate our clinical outcome and selected use of postoperative radiotherapy. Clinical materials and methods Between July 1990 and May 1995, nineteen patients [M : F = 12 : 7; age range: 21 to 71 years] with a spinal ependymoma were treated at University of Miami by the senior author. (BAG). Pre-operative MRI diagnosed the intraspinal tumor, and pathology reports demonstrated that each patient had a histologically confirmed ependymoma. At the time of diagnosis, the most common symptoms presented were pain (in 16 patients = 84.2%). The pattern of progression of clinical symptoms was directly related to the location of the tumor. Each patient had an MRI immediately after surgery, approximately 6 months post-operatively, and then annually. Results: All 19 patients underwent intradural microsurgical exploration with an attempted gross total resection (achieved in 16 patients = 79%) of the ependymoma through a posterior approach. Direct neural tissue stimulation halted further resection in 2 patients with questionable tumor margins. Radiation therapy was employed as a surgical adjunct in 3 patients (15.8%) because of possible residual tumor. All patients were followed up postoperatively for an average of 50.6 months (range 6 months to 6 years). All patients are surviving to date. Surgical resection of these tumors led to significant alleviation of pre-operative symptoms. There has been no radiographic evidence of tumor recurrence or growth in any patient to date. Conclusion: Surgical resection of spinal ependymoma leads to significant improvement of pre-operative symptom. Surgical removal alone, with an attempt to grossly resect the tumor

  18. The impact of routine whole spine MRI screening in the evaluation of spinal degenerative diseases.

    Science.gov (United States)

    Kanna, Rishi Mugesh; Kamal, Younis; Mahesh, Anupama; Venugopal, Prakash; Shetty, Ajoy Prasad; Rajasekaran, S

    2017-08-01

    Magnetic resonance imaging (MRI) of the spine is a sensitive investigation, which not only provides detailed images of the spinal column but also adjacent spinal regions and para-vertebral organ systems. Such incidental findings (IF) can be asymptomatic but significant. The efficacy of whole spine T2 sagittal screening in providing additional information has been demonstrated in several spinal diseases but its routine use in patients with spinal degenerative diseases has not been studied. A review of 1486 consecutive T2w whole spine screening MRI performed for cervical, thoracic or lumbar spinal imaging for degenerative diseases, was performed to document the incidence and significance of asymptomatic IF in the spinal and extra-spinal regions. 236 (15.88%) patients had IF with a M:F ratio of 102:134 and the mean age being 50.3 years. Of these, spinal IF was observed in 122 (51.7%-Group A) while extra-spinal IF was present in 114 (48.3%-Group B). In Group A, 84 patients had IF in the vertebral column and 38 patients had IF in the spinal cord. IF within the spine included vertebral haemangioma (n = 60, 4.5%), diffuse vertebral marrow changes (n = 18, 1.2%), vertebral metastasis (n = 2), incidental cord myelopathy (n = 21), intradural tumour (n = 7), and others. 33 patients required surgical intervention of the IF (2.2%). In Group B, pelvic IF were most prevalent (n = 79, 5.3%) followed by retro-peritoneal abdominal IF in 22 (1.48%) and intra-cranial IF in 9 (0.60%). 32 (2.1%) of these pathologies required further specialist medical or surgical evaluation. Routine T2 whole spine screening MRI identified 15.8% IF of the spinal and extra-spinal regions. 65 patients (4.3%) required either spine surgical intervention or other specialist care. Considering the potential advantages in identifying significant IF and the minimal extra time spent to perform whole spine screening, its application can be considered to be incorporated in routine imaging of spinal

  19. Microdeletion del(22(q12.2 encompassing the facial development-associated gene, MN1 (meningioma 1 in a child with Pierre-Robin sequence (including cleft palate and neurofibromatosis 2 (NF2: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Davidson Tom B

    2012-03-01

    Full Text Available Abstract Background Pierre-Robin sequence (PRS is defined by micro- and/or retrognathia, glossoptosis and cleft soft palate, either caused by deformational defect or part of a malformation syndrome. Neurofibromatosis type 2 (NF2 is an autosomal dominant syndrome caused by mutations in the NF2 gene on chromosome 22q12.2. NF2 is characterized by bilateral vestibular schwannomas, spinal cord schwannomas, meningiomas and ependymomas, and juvenile cataracts. To date, NF2 and PRS have not been described together in the same patient. Case presentation We report a female with PRS (micrognathia, cleft palate, microcephaly, ocular hypertelorism, mental retardation and bilateral hearing loss, who at age 15 was also diagnosed with severe NF2 (bilateral cerebellopontine schwannomas and multiple extramedullary/intradural spine tumors. This is the first published report of an individual with both diagnosed PRS and NF2. High resolution karyotype revealed 46, XX, del(22(q12.1q12.3, FISH confirmed a deletion encompassing NF2, and chromosomal microarray identified a 3,693 kb deletion encompassing multiple genes including NF2 and MN1 (meningioma 1. Five additional patients with craniofacial dysmorphism and deletion in chromosome 22-adjacent-to or containing NF2 were identified in PubMed and the DECIPHER clinical chromosomal database. Their shared chromosomal deletion encompassed MN1, PITPNB and TTC28. MN1, initially cloned from a patient with meningioma, is an oncogene in murine hematopoiesis and participates as a fusion gene (TEL/MN1 in human myeloid leukemias. Interestingly, Mn1-haploinsufficient mice have abnormal skull development and secondary cleft palate. Additionally, Mn1 regulates maturation and function of calvarial osteoblasts and is an upstream regulator of Tbx22, a gene associated with murine and human cleft palate. This suggests that deletion of MN1 in the six patients we describe may be causally linked to their cleft palates and/or craniofacial

  20. Keyhole interlaminar dorsal rhizotomy for spastic diplegia in cerebral palsy.

    Science.gov (United States)

    Sindou, Marc; Georgoulis, George

    2015-07-01

    The efficiency and safety of dorsal rhizotomies for cerebral palsy lie in the accuracy of radicular identification together with selectivity of root sectioning. Two different exposures are currently in use. The first is extended laminotomy/laminectomy from the upper lumbar level to the sacrum, which allows accurate identification of all L2-S2 roots/rootlets. The second is limited laminotomy exposing the conus/cauda equina at the thoracolumbar junction; this less invasive method limits accessibility to the roots. To optimize the accuracy and selectivity while minimizing invasiveness, the authors developed a tailored interlaminar procedure targeting the radicular levels involved in the harmful components of spasticity directly and individually. Six patients with spastic diplegia at different levels of the Gross Motor Functional Classification System were selected. In each patient, two to three interlaminar spaces, preselected according to planning, were enlarged in the "keyhole" fashion, respecting the spinous processes and interspinous ligaments. Ventral root stimulation identified the radicular level. Dorsal root stimulation evaluated its implication in the hyperactive segmental circuits, helping quantify the percentage of rootlets to be cut. There were neither wound-related nor general complications. At 1 year of follow-up, X-ray examination did not reveal kyphosis or instability. In all children, the excess of spasticity was reduced. The Ashworth score decreased from 3.2 on average to 0.6 postoperatively (range: 2-4 to 0-2). Regarding the functional status at 1 year of follow-up for the three ambulatory children, the Gillette ability-to-walk score increased from 3/10 on average to 7.3/10 postoperatively (range: 2-4 to 7-8). For the three non-ambulatory children, abnormal postures, painful contractures and ease of care were much improved. Keyhole interlaminar dorsal rhizotomy (KIDr) offers direct intradural access to each of the ventral/dorsal roots, thus

  1. At-Risk Phenotype of Neurofibromatose-1 Patients: A Multicentre Case-Control Study

    Directory of Open Access Journals (Sweden)

    Ferkal Salah

    2011-07-01

    Full Text Available Abstract Objectives To assess associations between subcutaneous neurofibromas (SC-NFs and internal neurofibromas in patients with neurofibromatosis type 1 (NF-1 and to determine whether the association between SC-NFs and peripheral neuropathy was ascribable to internal neurofibromas. Patients and methods Prospective multicentre case-control study. Between 2005 and 2008, 110 NF-1 adults having two or more SC-NFs were individually matched for age, sex and hospital with 110 controls who had no SC-NF. Patients underwent standardized MRI of the spinal cord, nerve roots and sciatic nerves and an electrophysiological study. Analyses used adjusted multinomial logistic regression (ORa to estimate the risk of the presence of internal neurofibromas or peripheral neuropathies associated with patients presented 2 to 9 SC-NFs, at least 10 SC-NFs as compared to patients without any (referential category. Results Cases had a mean age of 41 (± 13 years; 85 (80% had two to nine SC-NFs and 21 (19% at least ten SC-NFs. SC-NFs were more strongly associated with internal neurofibromas in patients with ten or more SC-NFs than in patients with fewer NF-SCs (e.g., sciatic nerve, aOR = 29.1 [8.5 to 100] vs. 4.3 [2.1 to 9.0]. The association with SC-NFs was stronger for diffuse, intradural, and > 3 cm internal neurofibromas than with other internal neurofibromas. Axonal neuropathy with slowed conduction velocities (SCV was more strongly associated with having at least ten SC-NFs (aOR = 29.9, 5.5 to 162.3 than with having fewer SC-NFs (aOR = 4.4, 0.9 to 22.0. Bivariate analyses showed that the association between axonal neuropathy with SCV and sciatic neurofibromas was mediated by the association between SC-NFs and sciatic neurofibromas. Conclusion The at-risk phenotype of NF-1 patients (i.e. NF-1 patients with SC-NFs is ascribable to associations linking SC-NFs to internal neurofibromas at risk for malignant transformation and to axonal neuropathies with slowed

  2. Sole stenting treatment for small wide-necked saccular intracranial aneurysms:a clinical therapeutic analysis

    International Nuclear Information System (INIS)

    Cheng Jiyong; Hong Bo; Xu Yi; Huang Qinghai; Yang Pengfei; Zhao Wenyuan; Liu Jianmin

    2010-01-01

    Objective: To discuss the feasibility, safety and efficacy of sole stenting technique for the treatment of small wide-necked saccular intracranial aneurysms. Methods: Between February 2001 and November 2009, 36 consecutive patients with small wide-necked saccular intracranial aneurysms (18 males and 18 females, aged 19-75 years,with a mean age of 52.3 years) were treated with stenting technique only. Of 36 patients, the Grade 0 of Hunt and Hess classification was seen in 22,Grade I in 8, Grade II in 5 and Grade III in 1. The aneurysmal diameter ranged from 1.8 mm to 5.0 mm,with a mean diameter of 3.6 mm. The aneurysms were located at the anterior communicating artery (n=1), posterior communicating artery (n=11), intradural paraclinoid internal carotid artery (n=18), basilar artery (n=1), anterior choroidal artery (n=4) or middle cerebral artery (n=1). The clinical manifestations, the angiographic findings and the follow-up observations were analyzed and the results were evaluated by means of the Modified Rankin Scale, magnetic resonance angiography and digital subtraction angiography. Results: Sole stenting treatment was performed in 36 patients with small wide-necked saccular intracranial aneurysms. A total of 37 stents were successfully delivered and deployed at the targeted location. Immediate post-procedural angiography showed that complete occlusion was obtained in one aneurysm, a sluggish intra-aneurysmal vortex motion in 3 aneurysms and a correction of the angle of the parent vessel in two cases, whereas no change was seen in the remaining 30 aneurysms. Angiographic follow-up was carried out in 22 of the 36 patients (61%) during a mean following-up period of 16 months (ranged from 3 to 59 months). The follow-up angiography showed that the complete occlusion was seen in 11 cases, narrowed aneurysmal neck with shrinkage of the aneurysm in 9 cases, narrowed aneurysmal neck only in 1 case, and shrinkage of the aneurysm only in 1 case.All the patients remained

  3. CT, MR and angiographic findings of hemangiopericytomas

    Energy Technology Data Exchange (ETDEWEB)

    Lim, Soo Mee; Lee, Ho Kyu; Shin, Ji Hoon; Kim, Jae Kyun; Kim, Dae Hong; Choi, Choong Gon; Suh, Dae Chul [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-07-01

    Hemangiopericytoma(HP) exhibits its pathologic findings different from those of meningioma or other angiomatous tumor; and its clinical behavior is unique and prognosis worse than other cases. We reviewed the CT, MR and angiographic findings of HPs and evaluated differential radiologic points of comparison between typical meningiomas and meningeal HPs. MR(n=16), CT(n=5) and angiographic imaging(n=10) were performed in 18 patients(M:F = 12:6, mean age: 45 years) with histologically proven primary HPs. We evaluated the imaging findings of HPs with respect to site, shape, size, signal intensity, enhancement characteristics, vascular signal voids, calcification, bony and adjacent sinus involvement, and angiographic findings. HPs were meningeal in 14 cases and nonmeningeal in four. Meningeal HPs were located in the parasagittal region(n=8), convexity(n=3), intradural extramedullary space(n=1), choroid plexus(n=1), and olfactory groove(n=1). Nonmeningeal HPs were located in the masticator space(n=2), paraspinal area(n=1) and supraclavicular area(n=1). The mean maximal dimension of tumors was about 5.4cm and their shape was papillary(n=8) or lobulated(n=7). MR images showed high(n=13) or iso(n=3) signal intensities on T2W1, and heterogeneity on T2W1(n=9). Vascular signal voids in the mass were seen in all cases, while in two cases, CT scanning showed nodular dense calcification. Bone destruction was present in six cases, but no hyperostosis was found. In five cases, the superior sagittal sinus was involved. Angiographic images revealed highly vascular masses supplied by the internal carotid artery(n=5), external carotid artery(n=8), descending scapular artery(n=1) and radiculomedullary artery(n=1), with delayed tumor blush during the capillary and venous phase in which there was no arteriovenous shunt. HP is one of the extra-axial tumors in which there is hypervascularity, aggressive bony destruction arising in the meningeal and extrameningeal area, and heterogeneous high

  4. Dose–Volume Relationships Associated With Temporal Lobe Radiation Necrosis After Skull Base Proton Beam Therapy

    Energy Technology Data Exchange (ETDEWEB)

    McDonald, Mark W., E-mail: markmcdonaldmd@gmail.com [Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana (United States); Indiana University Health Proton Therapy Center, Bloomington, Indiana (United States); Linton, Okechukwu R. [Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana (United States); Calley, Cynthia S.J. [Department of Biostatistics, Indiana University School of Medicine, Indianapolis, Indiana (United States)

    2015-02-01

    Purpose: We evaluated patient and treatment parameters correlated with development of temporal lobe radiation necrosis. Methods and Materials: This was a retrospective analysis of a cohort of 66 patients treated for skull base chordoma, chondrosarcoma, adenoid cystic carcinoma, or sinonasal malignancies between 2005 and 2012, who had at least 6 months of clinical and radiographic follow-up. The median radiation dose was 75.6 Gy (relative biological effectiveness [RBE]). Analyzed factors included gender, age, hypertension, diabetes, smoking status, use of chemotherapy, and the absolute dose:volume data for both the right and left temporal lobes, considered separately. A generalized estimating equation (GEE) regression analysis evaluated potential predictors of radiation necrosis, and the median effective concentration (EC50) model estimated dose–volume parameters associated with radiation necrosis. Results: Median follow-up time was 31 months (range 6-96 months) and was 34 months in patients who were alive. The Kaplan-Meier estimate of overall survival at 3 years was 84.9%. The 3-year estimate of any grade temporal lobe radiation necrosis was 12.4%, and for grade 2 or higher radiation necrosis was 5.7%. On multivariate GEE, only dose–volume relationships were associated with the risk of radiation necrosis. In the EC50 model, all dose levels from 10 to 70 Gy (RBE) were highly correlated with radiation necrosis, with a 15% 3-year risk of any-grade temporal lobe radiation necrosis when the absolute volume of a temporal lobe receiving 60 Gy (RBE) (aV60) exceeded 5.5 cm{sup 3}, or aV70 > 1.7 cm{sup 3}. Conclusions: Dose–volume parameters are highly correlated with the risk of developing temporal lobe radiation necrosis. In this study the risk of radiation necrosis increased sharply when the temporal lobe aV60 exceeded 5.5 cm{sup 3} or aV70 > 1.7 cm{sup 3}. Treatment planning goals should include constraints on the volume of temporal lobes receiving

  5. Helical tomotherapy. Experiences of the first 150 patients in Heidelberg

    Energy Technology Data Exchange (ETDEWEB)

    Sterzing, F.; Schubert, K.; Sroka-Perez, G.; Kalz, J.; Debus, J.; Herfarth, K. [Dept. of Radiation Oncology, Univ. of Heidelberg (Germany)

    2008-01-15

    Background and purpose: helical tomotherapy was introduced into clinical routine at the Department of Radiation Oncology, University Hospital of Heidelberg, Germany, in July 2006. This report is intended to describe the experience with the first 150 patients treated with helical tomotherapy. Patient selection, time effort, handling of daily image guidance with megavoltage (MV) CT, and quality of radiation plans shall be assessed. Patients and methods: between July 2006 and May 2007, 150 patients were treated with helical tomotherapy in the University Hospital of Heidelberg. Mean age was 60 years with a minimum of 30 years and a maximum of 85 years. 79 of these patients received radiotherapy as a part of multimodal treatment pre- or postoperatively, 17 patients received treatment as a combined radiochemotherapy. 76% were treated with curative intent. Radiotherapy sites were central nervous system (n = 7), head and neck (n = 28), thoracic (n = 37), abdominal (n = 58) and skeletal system (n = 20). Most common tumor entities were prostate cancer (n = 28), breast cancer (n = 17), gastrointestinal tumors (n = 19), pharyngeal carcinoma (n = 14), lymphoma (n = 13), metastatic disease (bone n = 14, liver n = 6, lung n = 4, lymph node n = 2), sarcoma (n = 8), malignant pleural mesothelioma (n = 5), ovarian cancer treated with whole abdominal irradiation (n = 4), lung cancer (n = 3), skin malignancies (n = 3), chordoma (n = 2), meningioma (n = 2), one ependymoma and one medulloblastoma treated with craniospinal axis irradiation (n = 2), and others (n = 4). Nine patients were treated with single-fraction radiosurgery, nine with image-guided spinal reirradiation, and twelve patients were treated at multiple targets simultaneously. A pretreatment MV-CT scan was performed in 98.2% of the 3,026 fractions applied. After matching with the kilovoltage planning CT, corrections for translations and rotation around longitudinal axis (roll) were done. Results: mean time on table was 24

  6. SU-F-T-124: Radiation Biological Equivalent Presentations OfLEM-1 and MKM Approaches in the Carbon-Ion Radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Hsi, W; Jiang, G; Sheng, Y [Shanghai Proton and Heavy-Ion Center, Shanghai, Shanghai (China)

    2016-06-15

    Purpose: To study the correlations of the radiation biological equivalent doses (BED) along depth and lateral distance between LEM-1 and MKM approaches. Methods: In NIRS-MKM (Microdosimetric Kinetic Model) approach, the prescribed BED, referred as C-Eq, doses aims to present the relative biological effectiveness (RBE) for different energies of carbon-ions on a fixed 10% survival value of HCG cell with respect to convention X-ray. Instead of a fixed 10% survival, the BED doses of LEM-1 (Local Effect Model) approach, referred as X-Eq, aims to present the RBE over the whole survival curve of chordoma-like cell with alpha/beta ratio of 2.0. The relationship of physical doses as a function of C-Eq and X-Eq doses were investigated along depth and lateral distance for various sizes of cubic targets in water irradiated by carbon-ions. Results: At the center of each cubic target, the trends between physical and C-Eq or X-Eq doses can be described by a linear and 2nd order polynomial functions, respectively. Using fit functions can then calculate a scaling factor between C-Eq and X-Eq doses to have similar physical doses. With equalized C-Eq and X-Eq doses at the depth of target center, over- and under-estimated X-Eq to C-Eq are seen for depths before and after the target center, respectively. Near the distal edge along depth, sharp rising of RBE value is observed for X-Eq, but sharp dropping of RBE value is observed for C-Eq. For lateral locations near and just outside 50% dose level, sharp raising of RBE value is also seen for X-Eq, while only minor increasing with fast dropping for C-Eq. Conclusion: An analytical function to model the differences between the CEq and X-Eq doses along depth and lateral distance need to further investigated to explain varied clinic outcome of specific cancers using two different approaches to calculated BED doses.

  7. Impact of tissue specific parameters on the predition of the biological effectiveness for treatment planning in ion beam therapy

    Energy Technology Data Exchange (ETDEWEB)

    Gruen, Rebecca Antonia

    2014-06-03

    Treatment planning in ion beam therapy requires a reliable estimation of the relative biological effectiveness (RBE) of the irradiated tissue. For the pilot project at GSI Helmholtzzentrum fuer Schwerionenforschung GmbH and at other European ion beam therapy centers RBE prediction is based on a biophysical model, the Local Effect Model (LEM). The model version in use, LEM I, is optimized to give a reliable estimation of RBE in the target volume for carbon ion irradiation. However, systematic deviations are observed for the entrance channel of carbon ions and in general for lighter ions. Thus, the LEM has been continuously developed to improve accuracy. The recent version LEM IV has proven to better describe in-vitro cell experiments. Thus, for the clinical application of LEM IV it is of interest to analyze potential differences compared to LEM I under treatment-like conditions. The systematic analysis presented in this work is aiming at the comparison of RBE-weighted doses resulting from different approaches and model versions for protons and carbon ions. This will facilitate the assessment of consequences for clinical application and the interpretation of clinical results from different institutions. In the course of this thesis it has been shown that the RBE-weighted doses predicted on the basis of LEM IV for typical situations representing chordoma treatments differ on average by less than 10 % to those based on LEM I and thus also allow a consistent interpretation of the clinical results. At Japanese ion beam therapy centers the RBE is estimated using their clinical experience from neutron therapy in combination with in-vitro measurements for carbon ions (HIMAC approach). The methods presented in this work allow direct comparison of the HIMAC approach and the LEM and thus of the clinical results obtained at Japanese and European ion beam therapy centers. Furthermore, the sensitivity of the RBE on the model parameters was evaluated. Among all parameters the

  8. D-IMRT verification with a 2D pixel ionization chamber: dosimetric and clinical results in head and neck cancer

    Science.gov (United States)

    Stasi, M.; Giordanengo, S.; Cirio, R.; Boriano, A.; Bourhaleb, F.; Cornelius, I.; Donetti, M.; Garelli, E.; Gomola, I.; Marchetto, F.; Porzio, M.; Sanz Freire, C. J.; Sardo, A.; Peroni, C.

    2005-10-01

    Dynamic intensity-modulated radiotherapy (D-IMRT) using the sliding-window technique is currently applied for selected treatments of head and neck cancer at Institute for Cancer Research and Treatment of Candiolo (Turin, Italy). In the present work, a PiXel-segmented ionization Chamber (PXC) has been used for the verification of 19 fields used for four different head and neck cancers. The device consists of a 32 × 32 matrix of 1024 parallel-plate ionization chambers arranged in a square of 24 × 24 cm2 area. Each chamber has 0.4 cm diameter and 0.55 cm height; a distance of 0.75 cm separates the centre of adjacent chambers. The sensitive volume of each single ionization chamber is 0.07 cm3. Each of the 1024 independent ionization chambers is read out with a custom microelectronics chip. The output factors in water obtained with the PXC at a depth of 10 cm were compared to other detectors and the maximum difference was 1.9% for field sizes down to 3 × 3 cm2. Beam profiles for different field dimensions were measured with the PXC and two other types of ionization chambers; the maximum distance to agreement (DTA) in the 20-80% penumbra region of a 3 × 3 cm2 field was 0.09 cm. The leaf speed of the multileaf collimator was varied between 0.07 and 2 cm s-1 and the detector response was constant to better than 0.6%. The behaviour of the PXC was measured while varying the dose rate between 0.21 and 1.21 Gy min-1; the mean difference was 0.50% and the maximum difference was 0.96%. Using fields obtained with an enhanced dynamic wedge and a staircase-like (step) IMRT field, the PXC has been tested for simple 1D modulated beams; comparison with film gave a maximum DTA of 0.12 cm. The PXC was then used to check four different IMRT plans for head and neck cancer treatment: cervical chordoma, parotid, ethmoid and skull base. In the comparison of the PXC versus film and PXC versus treatment planning system, the number of pixels with γ parameter <=1 was 97.7% and 97

  9. SU-E-P-06: A Novel Hybrid Planning Approach to Allow More Patients Benefited by the Intensity Modulated Proton Therapy

    Energy Technology Data Exchange (ETDEWEB)

    Jiang, S [UT MD Anderson Cancer Center, Tianjin Medical University Cancer Instit, Houston, TX (United States); Liao, L [UT MD Anderson Cancer Center, University of Houston, Houston, TX (United States); Li, Y [Varian Medical Systems, Houston, TX (United States); Wang, X; Sahoo, N; Liao, Z; Grosshans, D; Frank, S [MD Anderson Cancer Center, Houston, TX (United States); Li, H [M.D. Anderson Cancer Center, Houston, TX (United States); Zhu, X; Chang, J; Zhang, X [UT MD Anderson Cancer Center, Houston, TX (United States); Gillin, M [MD Anderson Cancer Ctr., Houston, TX (United States); Hojo, Y [UT MD Anderson cancer center, Houston, TX (United States); Sun, J [UT MD Anderson cancer center, Tianjin Medical University Cancer Institute a, Houston, TX (United States)

    2014-06-01

    Purpose: We report a hybrid scattering and scanning beam delivery approach, termed as (HimpsPT), which demonstrated that majority IMPT delivery can be potentially replaced with hybrid IMPT and PSPT delivery with similar or better plan quality. Methods: Three representative clinical cases, including head and neck (HN), skull base chordoma (CNS) and lung cancer, treated in MDACC Proton Therapy Center with IMPT were retrospectively redesigned using HimpsPT. The PSPT plans are designed with the same prescriptions as those of IMPT plans. The whole treatment can be delivered by either alternating or sequential PSPT and IMPT delivery. The dosimetric data and dose distributions of HimpsPT plans are compared with those of IMPT plans. We also performed a worst-case robust analysis for all plans. Results: The target coverages for all cases are comparable. For the HN case, the mean dose of esophagus larynx, left parotid and right submandibular, oral cavity V20, the max dose of cord is 18.0, 36.1, 23.6, 47.2, 0.1, 31.7 Gy in HimpsPT plan, and 25.5, 33.8, 24.9, 49.1, 0.2, 33.8 Gy in IMPT plan. For the lung case, the lung V5, V20, V30, mean lung dose, heart V40, esophagus V70, cord maximum dose are 50.5%, 37.0%, 31.7%, 21.3 Gy, 7.2%, 4.9%, 35.5 Gy in HimpsPT plan, and 55.4%, 36.7%, 30.1%, 21.3 Gy, 7.7%, 8.4%, 36.8Gy in IMPT plans. For the CNS case, brainstem maximum dose is 50.5 Gy in HimpsPT plan and 55.4 Gy in IMPT plan due to sharp penumbra offered by the aperture of the PSPT plan in HimpsPT technique. Conclusion: For majority disease sites, the dosimetric advantage of IMPT technique can be achieved by the hybrid PSPT and IMPT technique, which enables the centers equipped with both scattering and scanning beam facilities to treat more patients which can be benefited by the scanning beam.

  10. Impact of tissue specific parameters on the predition of the biological effectiveness for treatment planning in ion beam therapy

    International Nuclear Information System (INIS)

    Gruen, Rebecca Antonia

    2014-01-01

    Treatment planning in ion beam therapy requires a reliable estimation of the relative biological effectiveness (RBE) of the irradiated tissue. For the pilot project at GSI Helmholtzzentrum fuer Schwerionenforschung GmbH and at other European ion beam therapy centers RBE prediction is based on a biophysical model, the Local Effect Model (LEM). The model version in use, LEM I, is optimized to give a reliable estimation of RBE in the target volume for carbon ion irradiation. However, systematic deviations are observed for the entrance channel of carbon ions and in general for lighter ions. Thus, the LEM has been continuously developed to improve accuracy. The recent version LEM IV has proven to better describe in-vitro cell experiments. Thus, for the clinical application of LEM IV it is of interest to analyze potential differences compared to LEM I under treatment-like conditions. The systematic analysis presented in this work is aiming at the comparison of RBE-weighted doses resulting from different approaches and model versions for protons and carbon ions. This will facilitate the assessment of consequences for clinical application and the interpretation of clinical results from different institutions. In the course of this thesis it has been shown that the RBE-weighted doses predicted on the basis of LEM IV for typical situations representing chordoma treatments differ on average by less than 10 % to those based on LEM I and thus also allow a consistent interpretation of the clinical results. At Japanese ion beam therapy centers the RBE is estimated using their clinical experience from neutron therapy in combination with in-vitro measurements for carbon ions (HIMAC approach). The methods presented in this work allow direct comparison of the HIMAC approach and the LEM and thus of the clinical results obtained at Japanese and European ion beam therapy centers. Furthermore, the sensitivity of the RBE on the model parameters was evaluated. Among all parameters the

  11. Use of recombinant human bone morphogenetic protein-2 as an adjunct for instrumented posterior arthrodesis in the occipital cervical region: An analysis of safety, efficacy, and dosing

    Directory of Open Access Journals (Sweden)

    D Kojo Hamilton

    2010-01-01

    Full Text Available Background: There have been few reports on the use of recombinant human bone morphogenetic protein (rhBMP-2 in posterior spine. However, no study has investigated the dosing, safety, and efficacy of its use in the posterior atlantoaxial, and/or craniovertebral junction. Recent case report of the cytokine-mediated inflammatory reaction, following off label use of rhBMP-2 as an adjunct for cervical fusion, particularly in complex cases, has increased concern about complications associated with the product. Objective: To assess the safety, efficacy, and dosing of rhBMP-2 as an adjunct for instrumented posterior atlantoaxial and/or craniovertebral junction arthrodesis. Materials and Methods: We included all patients treated by the senior author that included posterior atlantoaxial and/or craniovertebral junction instrumented fusion using rhBMP-2 from 2003 to 2008 with a minimum two year follow-up. Diagnosis, levels fused, rhBMP-2 dose, complications, and fusion were assessed. Results: Twenty three patients with a mean age of 60.9 years (range 4 - 89 years and an average follow-up of 45 months (range 27 to 84 months met inclusion criteria. The indications for surgery included, atlantoaxial instability (n = 16, basilar invagination (n = 6, and kyphoscoliosis (n = 1. The specific pathologic diagnosis included type 2 dens fracture (n = 7, complex C1 and C2 ring fracture (n = 2, chordoma (n = 2, degenerative/osteoporosis (n = 3, rheumatoid disease (n = 8, and pseudogout (n = 1. The average rhBMP-2 dose was 2.38 mg/level, with a total of 76 levels treated (average 3.3 levels, SD= 1.4 levels. There were no complications. During the most recent follow-up, all patients had achieved fusion. Conclusions: In a series of patients with complex pathology and/or rheumatoid arthritis, 100% fusion rate was achieved with adjunct use of rhBMP-2, with a safe and effective average rhBMP-2 dose of 2.38 mg per level.

  12. Including robustness in multi-criteria optimization for intensity-modulated proton therapy.

    Science.gov (United States)

    Chen, Wei; Unkelbach, Jan; Trofimov, Alexei; Madden, Thomas; Kooy, Hanne; Bortfeld, Thomas; Craft, David

    2012-02-07

    We present a method to include robustness in a multi-criteria optimization (MCO) framework for intensity-modulated proton therapy (IMPT). The approach allows one to simultaneously explore the trade-off between different objectives as well as the trade-off between robustness and nominal plan quality. In MCO, a database of plans each emphasizing different treatment planning objectives, is pre-computed to approximate the Pareto surface. An IMPT treatment plan that strikes the best balance between the different objectives can be selected by navigating on the Pareto surface. In our approach, robustness is integrated into MCO by adding robustified objectives and constraints to the MCO problem. Uncertainties (or errors) of the robust problem are modeled by pre-calculated dose-influence matrices for a nominal scenario and a number of pre-defined error scenarios (shifted patient positions, proton beam undershoot and overshoot). Objectives and constraints can be defined for the nominal scenario, thus characterizing nominal plan quality. A robustified objective represents the worst objective function value that can be realized for any of the error scenarios and thus provides a measure of plan robustness. The optimization method is based on a linear projection solver and is capable of handling large problem sizes resulting from a fine dose grid resolution, many scenarios, and a large number of proton pencil beams. A base-of-skull case is used to demonstrate the robust optimization method. It is demonstrated that the robust optimization method reduces the sensitivity of the treatment plan to setup and range errors to a degree that is not achieved by a safety margin approach. A chordoma case is analyzed in more detail to demonstrate the involved trade-offs between target underdose and brainstem sparing as well as robustness and nominal plan quality. The latter illustrates the advantage of MCO in the context of robust planning. For all cases examined, the robust optimization for

  13. Seguimiento radiológico en paciente con fibroma condromixoide del seno frontal: Diagnóstico diferencial, alternativas terapéuticas y revisión de la literatura Radiological follow-up of a patient with chondromyxoid fibroma of the frontal sinus: Differential diagnosis, therapeutic alternatives and a revision of the literature

    Directory of Open Access Journals (Sweden)

    I. Zubillaga Rodriguez

    2006-12-01

    Full Text Available Introducción. El fibroma condromixoide es un tumor benigno de crecimiento lento y poco frecuente. Su incidencia en la región craneofacial es rara, especialmente en el seno frontal. Objetivos. Presentamos el seguimiento a largo plazo de un paciente intervenido en relación a un fibroma condromixoide del seno frontal. Se discuten los distintos diagnósticos diferenciales y las alternativas terapéuticas. Discusión. Desde el punto de vista de anatomía patológica es necesario hacer el diagnóstico diferencial con condrosarcoma, mixosarcoma, mixoma y cordoma. Desde el punto de vista radiológico, el diagnóstico de fibroma condromixoide ha de ser considerado en toda lesión ósea aislada con expansión u osteolisis, márgenes lobulados y septos intralesionales. El tratamiento del fibroma condromixoide incluye principalmente curetaje o resección en bloque, mientras que la radioterapia aislada no se considera de primera elección. Conclusiones. La naturaleza benigna del fibroma condromixoide implica la importancia de evitar tratamientos radicales. El abordaje subcraneal se presenta como una alternativa válida para el tratamiento de lesiones localizadas en el seno frontal. Es fundamental un seguimiento clínico-radiológico adecuado para identificar precozmente posibles recidivas.Introduction. Chondromyxoid fibroma is a slow growing and rare benign bone tumor. Its location in the craniofacial skeleton is not common, especially in the frontal sinus. Objectives. We present a long-term follow-up of a patient with a frontal chondromyxoid fibroma who was operated four years ago. We discuss the various differential diagnoses and possible treatments. Discussion. The differential diagnosis includes chondrosarcoma, myxosarcoma, myxoma and chordoma. From the radiological point of view, chondromyxoid fibroma must be always considered as a diagnostic possibility when a solitary bone lesion appears with expansion or cortical osteolysis, lobulated margins and

  14. Myxoid Chondrosarcoma of the Sinonasal Cavity in a Child: a Case Report

    International Nuclear Information System (INIS)

    Kim, Yeo Ju; Im, Soo Ah; Lim, Gye Yeon; Chun, Ho Jong; Park, Hyun Jin; Kim, Min Sik; Choi, Yeong Jin

    2007-01-01

    Chondrosarcomas are malignant tumors of cartilage that rarely involve the sinonasal region, and myxoid chondrosarcoma is a rare histologic variant of chondrosarcoma that usually occurs in the soft tissue of extremities. Although several case reports and results of small series of chondrosarcomas in the sinonasal region in children are available, myxoid type chondrosarcoma is extremely rare. We recently experienced a case of low grade myxoid chondrosarcoma involving the sinonasal cavity in a 10-year-old boy, and here we report its radiologic-pathologic findings. In this case, chondroid calcification on CT and septal and marginal enhancement on MRI suggested a chondrosarcoma. Whole body PET-CT demonstrated no definite metastatic lesion and a low peak standardized uptake value primary tumor. However, no definite distinguishing imaging features were observed that distinguished low grade myxoid chondrosarcoma from conventional chondrosarcoma. hondrosarcomas are malignant mesenchymal tumors of cartilage, and usually involve the long bone and pelvis. Less than 10% of chondrosarcomas are found in the head and neck region. Chondrosarcomas can occur at any age, but the majority present between the 5th and 7th decades. Therefore, chondrosarcomas of the head and neck in children are rare; only about 13 cases of sinonasal chondrosarcoma in children have been reported. Myxoid chondrosarcoma is a rare histologic variant of chondrosarcoma, and is characterized by abundant chondroid matrix and malignant chondroblastic cells arranged in cords resembling chordoma. Myxoid chondrosarcomas are typically located in the limbs in older patients, and only rarely originate in the head and neck in children. To the best of our knowledge, only one case report of myxoid type chondrosarcoma in the sinonasal region in a child is available. We report a case of myxoid chondrosarcoma involving the sinonasal cavity in a child and describe its computed tomography (CT), magnetic resonance imaging (MRI

  15. Tratamento de dor em paciente com tumor sacral inoperável: relato de caso Tratamiento de dolor en paciente con tumor sacral inoperable: relato de caso Management of pain in patient with inoperable sacral tumor: case report

    Directory of Open Access Journals (Sweden)

    Juliano Rodrigues Gasparini

    2004-04-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: Diversas técnicas podem ser utilizadas no controle de dor em pacientes oncológicos. O objetivo deste relato é mostrar o uso de algumas medidas terapêuticas empregadas para tratar um paciente com quadro doloroso importante de difícil controle. RELATO DO CASO: Paciente do sexo masculino, 70 anos, com cordoma sacral e com possibilidade terapêutica muito difícil. Apresentava quadro de dor importante associado ao tumor. São relatadas diversas téc