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Sample records for intradural retroclival chordoma

  1. A unique presentation of retroclival chordoma.

    Directory of Open Access Journals (Sweden)

    Warakaulle D

    2002-10-01

    Full Text Available Chordomas are rare tumours which arise from remnants of the primitive notochord. They occur primarily in the sacrum, clivus and cervical regions. We report a case of retroclival chordoma which presented as an extradural haemorrhage following minor trauma. The underlying tumour was not apparent on imaging performed immediately following the event, and chordoma presenting in this manner has not previously been described in the literature. The tumour became apparent on subsequent imaging, and progressed despite surgical debulking and radiotherapy.

  2. Magnetic resonance imaging of the intradural prepontine chordoma mimicking an epidermoid cyst: Pathologic correlation

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    Cho, Hyoun; Yu, In Kyu; Kim, Seung Min; Kim, Han Kyu [Eulji Univ. Hospital, Daejeon (Korea, Republic of)

    2012-08-15

    Intracranial chordomas, originating from remnants of the primitive notochord, are extradural tumors arising mostly at the sphenooccipital synchondrosis in the clivus. We present an unusual case of intradural chordoma at the prepontine cistern, with parenchymal compressive invasion to the pons. It was excised subtotally, followed by a second operation due to the increasing remnant tumor size during 8 months. A differential diagnosis for intradural chordoma must be considered when the preoperative MRI features are not consistent with an epidermoid cyst if there are multiple fine enhancing lesions on enhanced magnetic resonance images and no bright signal intensity on diffusion weighted images. This report is concerned with the radiological findings in the intradural chordoma and the differential diagnosis focused on the epidermoid cyst.

  3. Magnetic resonance imaging of the intradural prepontine chordoma mimicking an epidermoid cyst: Pathologic correlation

    International Nuclear Information System (INIS)

    Cho, Hyoun; Yu, In Kyu; Kim, Seung Min; Kim, Han Kyu

    2012-01-01

    Intracranial chordomas, originating from remnants of the primitive notochord, are extradural tumors arising mostly at the sphenooccipital synchondrosis in the clivus. We present an unusual case of intradural chordoma at the prepontine cistern, with parenchymal compressive invasion to the pons. It was excised subtotally, followed by a second operation due to the increasing remnant tumor size during 8 months. A differential diagnosis for intradural chordoma must be considered when the preoperative MRI features are not consistent with an epidermoid cyst if there are multiple fine enhancing lesions on enhanced magnetic resonance images and no bright signal intensity on diffusion weighted images. This report is concerned with the radiological findings in the intradural chordoma and the differential diagnosis focused on the epidermoid cyst

  4. Chordoma

    International Nuclear Information System (INIS)

    Saxton, J.P.

    1981-01-01

    Nineteen patients with chordoma seen at M.D. Anderson Hospital from 1948 to 1976 received definitive treatment. Six patients presented with disease in the basisphenoid region, 2 with disease in the lumbar spine (vertebral area), and 11 with disease in the sacrococcygeal area. Nine patients were treated with a combination of surgery and postoperative radiation therapy, 6 received radiation therapy only, and 4 underwent surgery only. Although the number of patients studied is small, the results suggest that surgery only is not an effective means of treating this disease. Radiation therapy only produces palliation for large inoperable lesions, but excision followed by irradiation is the best treatment for securing prolonged local control

  5. Intradural lipoma

    International Nuclear Information System (INIS)

    Schubert, F.

    1996-01-01

    An intradural lipoma consists of a localized collection of fat within the intradural space of the spinal canal. Microscopically, spinal lipomas consist of a homogeneous mass of adult adipose tissue (rarely, brown fat or mixed yellow and brown fat) that exhibits no evidence of malignancy. The fat is divided into lobules by thick connective-tissue strands which may be associated with smooth muscle fibres. Striated muscle fibres, angiomatous elements, calcification and ossification are less common. A case of intradural lipoma is presented and it is shown how both computer tomography and magnetic resonance imaging (MRI) assisted in an accurate diagnosis of this pathology. MR is the imaging modality of choice as it allows to fully evaluate the extent of the lipoma and its relationship to the neural placode, spinal cord and roots of the cord and/or cauda equina. 4 refs., 5 figs

  6. 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.)

  7. Retroclival collections associated with abusive head trauma in children.

    Science.gov (United States)

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

    2014-12-01

    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.

  8. 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.)

  9. Clival chordomas

    International Nuclear Information System (INIS)

    Schamschula, R.G.; Soo, M.Y.S.

    1993-01-01

    Three cases of clival chordomas are reviewed and the findings are compared to those in the recent literature. Chordomas are rare neoplasms that arise from primitive notochord remnants. In all three cases, computed tomography was an excellent modality for demonstrating bone destruction, sequestra and calcification but inferior to magnetic resonance imaging (MRI) in demonstrating the soft tissue extent of the tumour. The multiplanar capability of MRI was found to be particularly useful for planning treatment. Vertebral angiography can demonstrate the tumours by vessel displacement, encasement and vascular staining. Two cases had angiography and demonstrated tumour blush. Pre-operative embolization was helpful in one case. 13 refs., 1 tab., 6 figs

  10. Cytodiagnosis of Sacral Chordoma

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    Saumya Shukla

    2014-12-01

    Full Text Available We report the cytological findings of a sacro-coccygeal chordoma in a 53 year male diagnosed preoperatively by guided fine needle aspiration cytology. The smears shows characteristic Physalliphorous cells in a metachromatic background of myxoid material entrapping cords of cuboidal cells. Differential diagnosis in cytology include conventional and myxoid chondrosarcoma, myxoid liposarcoma, myxoid malignant fibrous histiocytoma, metastatic mucinous carcinoma and myxo-papillary ependymoma. The distinguishing features between these neoplasms are discussed. Preoperative diagnosis of chordoma permits optimum planned surgery. Keywords: chordoma; myxoid; sacral.

  11. Lumbar spine chordoma

    Directory of Open Access Journals (Sweden)

    M.A. Hatem, M.B.Ch.B, MRes, LMCC

    2014-01-01

    Full Text Available Chordoma is a rare tumor arising from notochord remnants in the spine. It is slow-growing, which makes it difficult to diagnose and difficult to follow up after treatment. Typically, it occurs in the base of the skull and sacrococcygeal spine; it rarely occurs in other parts of the spine. CT-guided biopsy of a suspicious mass enabled diagnosis of lumbar spine chordoma.

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

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    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

  13. Primary Intradural Extraosseous Ewing's Sarcoma

    OpenAIRE

    Kim, Seok Won; Shin, Ho

    2009-01-01

    Ewing's sarcoma usually arises from skeletal bone, but rarely may have an extraskeletal origin. However, Ewing's sarcoma that originates around the spinal column, especially, the intradural extramedullary type is extremely rare. We report a rare case of primary intraspinal extraskeletal Ewing's sarcoma.

  14. Radiation therapy for chordomas

    International Nuclear Information System (INIS)

    Ikeda, Hajime; Takahashi, Takeo; Nakamura, Yuji; Niibe, Hideo

    1995-01-01

    Chordomas are slow-growing primary malignant bone tumors which originate from remnants of the fetal notochordal system. They are difficult to control by surgery alone. Four patients with chordomas treated with radiation therapy were studied, and the effectiveness of radiotherapy was evaluated. These 4 (3.8%) patients were among 106 patients with primary malignant bone tumors referred to us from 1959 to 1987. Primary sites were the sacrococcygeal region in three patients and the clivus in one. The patients' ages ranged from 51 to 75 years. The male : female ratio was 1 : 1. Patients received 48 to 60 Gy of radiation to the primary sites. Because the radiosensitivity of the tumors was low, the responses were poor. The duration of survival was 6, 33, 68, and 125 months. The cause of death in each case was local recurrence of tumor. As a result, a dose greater than 60 Gy is thought to be necessary for curative radiotherapy. Proton beam therapy seems to be best choice for chordomas in the clivus, and mixed-beam (proton and megavolt age X-ray) therapy or multiportal irradiation, which gives an ideal spatial dose distribution, seems to be most suitable for sacrococcygeal chordomas. (author)

  15. Cervical chordoma: a case report

    International Nuclear Information System (INIS)

    Romera, C.; Wiehoff, A.; Candela, V. P.; Perera, J.

    2002-01-01

    Chordomas, lesions that develop from notochordal remnants, can arise at any site ranging from the clivus to the sacrum: they represent 3% to 4% of all primary bone tumors. We present the cases of a 45-year-old man with cervical chordoma at the C2 level, the site least frequently reported in the literature. We provide the radiological findings resulting from cervical computed tomography and magnetic resonance imaging. (Author) 11 refs

  16. Thoracic chordoma: CT and MR findings

    International Nuclear Information System (INIS)

    Cha, Yoo Mi; Hwang, Hee Young; Kim, Sang Joon; Chung, Hyo Sun; Han, Heon

    1993-01-01

    Chordoma arising from the notochordal remnants is a rare primary bone tumor in the cervicosacral region and is even more unusual in the thoracic region. The authors experienced a case of thoracic chordoma and reports its CT and MR findings

  17. 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 ...

  18. Petrous apex chordoma - a case report

    International Nuclear Information System (INIS)

    Silveira, Claudio Regis S.; Barreto, Cristina Marques; Rossi, Luis Antonio; Michiloski, Custodio; Rotta, Jose Marcus; Almeida, Serguey Malaquias de

    2001-01-01

    Chordomas are rare neoplasms arising from notochordal remnants that persist along the axial skeleton. Intracranial chordomas occur more frequently in the midline. We describe a typical 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. (author)

  19. [Application of neuroendoscope in the treatment of skull base chordoma].

    Science.gov (United States)

    Zhang, Ya-Zhuo; Wang, Zong-Cheng; Zong, Xu-Yi; Wang, Xin-Sheng; Gui, Song-Bai; Zhao, Peng; Li, Chu-Zhong; He, Yue; Wang, Hong-Yun

    2011-07-05

    To further explore the application, approach, indication and prognosis of neuroendoscope treatment for skull base chordoma. A total of 101 patients of skull base chordoma were admitted at our hospital from May 2000 to April 2010. There were 59 males and 42 females. Their major clinical manifestations included headache, cranial nerve damage and dyspnea. They were classified according to the patterns of tumor growth: Type I (n = 13): tumor location at a single component of skull base, i. e. clivus or sphenoid sinus with intact cranial dura; Type II (n = 56): tumor involving more than two components of skull e. g clivus, sphenoid and nasal/oral cavity, etc. But there was no intracranial invasion; Type III (n = 32) : tumor extending widely and intradurally forming compression of brain stems and multiple cranial nerves. Based on the types of chordoma, different endoscopic approaches were employed, viz. transnasal, transoral, trans-subtemporal fossa and plus microsurgical craniotomy for staging in some complex cases. Among all patients, total resection was achieved (n = 19), subtotal (n = 58) and partial (n = 24). In partial resection cases, 16 cases were considered to be subtotal due to a second-stage operation. Most cases had conspicuous clinical improvements. Self-care recovery within one week post-operation accounted for 58.4%, two weeks 30.7%, one month 6.9% and more than one month 1.9%. Postoperative complications occurred in 13 cases (12.8%) and included CSF leakage (n = 4) cranial nerve palsy (n = 5), hemorrhagic nasal wounds (n = 3) and delayed intracranial hemorrhage (n = 1). All of these were cured or improved after an appropriate treatment. A follow-up of 6 - 60 months was conducted in 56 cases. Early detection and early treatment are crucial for achieving a better outcome in chordoma. Neuroendoscopic treatment plays an important role in managing those complicated cases. Precise endoscopic techniques plus different surgical approaches and staging procedures

  20. Primary Intradural Hemangiopericytoma With Intramedullary Invasion

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    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

  1. Hemorrhagic chondroid chordoma mimicking pituitary apoplexy

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    Lee, H.J.; Kalnin, A.J.; Holodny, A.I. [Dept. of Radiology, University Hospital, Newark, NJ (United States); Schulder, M.; Grigorian, A. [Dept. of Neurosurgery, University Hospital, Newark, NJ (United States); Sharer, L.R. [Dept. of Pathology, University Hospital, Newark, NJ (United States)

    1998-11-01

    We describe a hemorrhagic chondroid chordoma involving the sella turcica with suprasellar extension. The CT and MRI appearances mimiked a hemorrhagic pituitary adenoma. Chondroid chordoma is a variant composed of elements of both chordoma and cartilaginous tissue. An uncommon bone neoplasm, located almost exclusively in the spheno-occipital region, it is usually not considered in the differential diagnosis of a tumor with acute hemorrhage in the sellar region. We discuss the clinical and radiological characteristics which may allow one to differentiate chondroid chordoma from other tumors of this area. (orig.) With 3 figs., 9 refs.

  2. Intradural squamous cell carcinoma in the sacrum

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    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.

  3. Spinal intradural extraosseous Ewing’s sarcoma

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    Daniel Lachance

    2011-03-01

    Full Text Available Extraosseous Ewing’s sarcoma (EES involving the central nervous system is rare, but can be diagnosed and distinguished from other primitive neuroectodermal tumors (PNET by identification of the chromosomal translocation (11;22(q24;q12. We report EES arising from the spinal intradural extramedullary space, based on imaging, histopathological, and molecular data in two men, ages 50 and 60 years old and a review of the literature using PubMed (1970-2009. Reverse transcriptase polymerase chain reaction (RT-PCR identified the fusion product FL1-EWS. Multimodal therapy, including radiation and alternating chemotherapy including vincristine, cyclophosphamide, doxorubicin and ifosfamide and etoposide led to local tumor control and an initial, favorable therapeutic response. No systemic involvement was seen from the time of diagnosis to the time of last follow-up (26 months or death (4 years. This report confirms that EES is not confined to the earliest decades of life, and like its rare occurrence as an extra-axial meningeal based mass intracranially, can occasionally present as an intradural mass in the spinal canal without evidence of systemic tumor. Gross total resection followed by multimodal therapy may provide for extended progression free and overall survival.

  4. 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.)

  5. 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.

  6. Chordoma

    African Journals Online (AJOL)

    is bound to result in relief of pain and may even prolong life expectancy. .... regrowth of the tumour, and with the patients all asympto- matic. ..... Ten days later the tumour was much ... visible, his speech had improved, and he was back to doing.

  7. Frequent activation of EGFR in advanced chordomas

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    Dewaele Barbara

    2011-07-01

    Full Text Available Abstract Background Chordomas are rare neoplasms, arising from notochordal remnants in the midline skeletal axis, for which the current treatment is limited to surgery and radiotherapy. Recent reports suggest that receptor tyrosine kinases (RTK might be essential for the survival or proliferation of chordoma cells, providing a rationale for RTK targeted therapy. Nevertheless, the reported data are conflicting, most likely due to the assorted tumor specimens used for the studies and the heterogeneous methodological approaches. In the present study, we performed a comprehensive characterization of this rare entity using a wide range of assays in search for relevant therapeutic targets. Methods Histopathological features of 42 chordoma specimens, 21 primary and 21 advanced, were assessed by immunohistochemistry and fluorescent in situ hybridization (FISH using PDGFRB, CSF1R, and EGFR probes. Twenty-two of these cases, for which frozen material was available (nine primary and 13 advanced tumors, were selectively analyzed using the whole-genome 4.3 K TK-CGH-array, phospho-kinase antibody array or Western immunoblotting. The study was supplemented by direct sequencing of KIT, PDGFRB, CSF1R and EGFR. Results We demonstrated that EGFR is frequently and the most significantly activated RTK in chordomas. Furthermore, concurrent to EGFR activation, the tumors commonly reveal co-activation of alternative RTK. The consistent activation of AKT, the frequent loss of the tumor suppressor PTEN allele, the recurrent activation of upstream RTK and of downstream effectors like p70S6K and mTOR, all indicate the PI3K/AKT pathway as an important mediator of transformation in chordomas. Conclusions Given the complexity of the signaling in chordomas, combined treatment regimens targeting multiple RTK and downstream effectors are likely to be the most effective in these tumors. Personalized therapy with careful selection of the patients, based on the molecular profile of

  8. The top 50 cited articles on chordomas.

    Science.gov (United States)

    Ikpeze, Tochukwu; Mesfin, Addisu

    2018-03-01

    Chordomas are rare malignant primary tumors of the spine. In the mobile spine and sacrum an en-bloc resection is associated with decreased rates of recurrence. Our objective was to identify the top cited articles in chordoma research and to further analyze characteristics of these articles. In March 2017, we used ISI Web of Science (v5.11, Thomas Reuter, Philadelphia, Pennsylvania, USA) to search for the following key word: "chordoma". Articles were searched from 1900 to 2017. Articles were ranked based on number of citations. The results were evaluated to determine articles most clinically relevant to the management of chordomas. The top 50 articles that met the search criteria were further characterized on the basis of: title, author, citation density, journal of publication, year (and decade) of publication, institution and country of origin and paper topic. A total of 1,043 articles matched the search criteria. The most influential 50 articles were cited 65 to 290 times. The articles were published between 1926 and 2012, and all articles were published in English. Thirty-three publications (66%) originated from the United States and seven (14%) from Italy. Cancer accounted for the most frequent (n=9) destination journal followed by Journal of Bone and Joint Surgery (n=4). A total of 41 institutions contributed to the top 50 articles. The most common article types were: clinical 44% (n=22), papers that combined clinical and pathology findings 18% (n=9) and basic science research 14% (n=7). The top 50 cited articles on chordomas are predominantly clinical papers, arising from the United States and most frequently published in Cancer and Journal of Bone and Joint Surgery .

  9. Combined Therapy for Distant Metastasis of Sacral Chordoma

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    Birol Özkal

    2015-01-01

    Full Text Available Chordomas are known as rare primary malign tumours that have formed from primitive notochord remains. Sacral chordomas grow slowly but locally and aggressively. Chordomas are locally invasive and have low tendency to metastasis and have a poor prognosis in long-term follow-up. Metastasis may be seen in a rate of 5–40% of the chordomas. Metastasis of chordomas is common in liver, lung, lymph nodes, peritoneum, and brain. The treatment approaches, including surgery, have been discussed in the literature before. Susceptibility to radiotherapy and chemotherapy is controversial in these tumours. The success of surgical treatment affects survival directly. In this report, we will report a sacral chordoma case in which an intraperitoneal distant metastasis occurred and discuss the surgical approach.

  10. 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)

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

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    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.

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

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    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.)

  13. 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.)

  14. High-resolution whole-genome analysis of skull base chordomas implicates FHIT loss in chordoma pathogenesis.

    Science.gov (United States)

    Diaz, Roberto Jose; Guduk, Mustafa; Romagnuolo, Rocco; Smith, Christian A; Northcott, Paul; Shih, David; Berisha, Fitim; Flanagan, Adrienne; Munoz, David G; Cusimano, Michael D; Pamir, M Necmettin; Rutka, James T

    2012-09-01

    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. 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.

  16. 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)

  17. Sacrococcygeal chordoma: MR imaging in 30 patients

    Energy Technology Data Exchange (ETDEWEB)

    Sung, Mi Sook; Chung, Myung Hee [Catholic University of Korea, Holy Family Hospital, Department of Radiology, Pucheon (Korea); Lee, Gyung Kyu; Kang, Heung Sik [Seoul National University Hospital, Department of Radiology, Seoul (Korea); Kwon, Soon Tae [Chungnam University Hospital, Department of Radiology, Taejun (Korea); Park, Jin Gyoon [Chunnam University Hospital, Department of Radiology, Kwangju (Korea); Suh, Jin Suk [Yonsei University, Severans Hospital, Department of Radiology, Seoul (Korea); Cho, Gil Ho [Yeungnam University Hospital, Department of Radiology, Taegu (Korea); Lee, Sung Moon [Kaemyung University Hospital, Department of Radiology, Taeku (Korea); Resnick, Donald [VA Medical Center, Department of Radiology, San Diego, CA (United States)

    2005-02-01

    To evaluate MR imaging of sacrococcygeal chordoma. Thirty patients (age range 22-80 years) underwent MR imaging for the diagnosis and preoperative evaluation of sacrococcygeal chordomas. Eight patients had follow-up MR examination after treatment. The MR images were performed with T1- and T2-weighted imaging, and gadolinium (Gd)-enhanced imaging. The MR images were analyzed for the signal intensity, enhancing pattern, tumor size, growth pattern of the soft tissue component, and tumor extension. T1-weighted images showed low signal masses with foci of high signal intensity in 73% of cases. Tumors enhanced in a variety of patterns after the administration of Gd. Soft tissue masses extending anteriorly were seen in all cases with posterior extension in 77% of cases. The posterior masses involved the surrounding muscles and extended toward the greater sciatic notch, appearing with pseudopodia (87%). Sacroiliac joints were involved in 23% of cases. Four lesions showed intraspinal extension and involvement of the posterior spinal muscles above the level of bony involvement. In 6 patients recurrent tumors were found at or around the surgical margin of the tumor 6 months to 5 years after resection of the sacral tumor. In two of the patients, nodular metastases to the pelvic bones and femur were found 1-4 years after initial examination. In conclusion, MR imaging is useful in the diagnosis and preoperative assessment of sacrococcygeal chordoma. Characteristic findings included sacral mass with heterogeneously high signal intensity with crisscrossing septa on long-repetition-time imaging, well-encapsulated pseudopodia-like or lobulated appearance, and gluteal muscle infiltration. Follow-up MR imaging is helpful to assess for recurrent or metastatic lesions of chordomas. (orig.)

  18. Magnetic resonance imaging (MRI) of intracranial chordomas

    Energy Technology Data Exchange (ETDEWEB)

    Fukuda, Teruo; Inoue, Yuichi; Shakudo, Miyuki and others

    1988-03-01

    MR images of 5 patients with intracranial chordoma were evaluated and compared with those of other clival lesions (1 clival osteomyelitis, 1 metastatic clival tumor, 3 clival meningiomas). The MR examination was performed using a 0.5 T superconductive magnet, with approximately 10 mm section thickness, one average and a 256 x 256 matrix. T1 weighted images were obtainned by inversion recovery (IR) with TR 2100 - 2500 msec, TI 600 msec and TE 40 msec. T2 weighted images were obtained by spin echo pulse sequence with TR 1800 - 2500 msec and TE 120 msec (long SE). In several cases, the spin echo pulse sequences with TR 1000 msec and TE 40 msec (short SE) were also done. Multiplaned images were obtained. Four of 5 intracranial chordomas were low in intensity compared to cerebral gray matter on T1 weighted images, and all of 5 chordomas were as high in intensity as cerebrospinal fluid or higher than that of cerebrospinal fluid on T2 weighted images. Clival fatty marrow is high intensity on T1 weighted images. Clival involvement by a tumor was a clearly demonstrated as disappearance of this high intensity in all cases. In two cases, the tumor extended to the retropharyngeal space and this was detected clearly on short SE image. Although clival fatty marrow was disappeared, osteomyelitis and metastatic tumor in clivus were iso-intense to cerebral gray matter on both T1 and T2 weighted images. All of 3 clival meningiomas showed iso-intensity to cerebral gray matter on T1 weighted images and slightly high intensity to brain on T2 weighted images, and clival fatty marrow was normal in all 3 cases. Although our experiences are limited in number, intracranial chordoma appeared to be differentiated from other clival lesions.

  19. Case report 544: Metastatic chordoma to humeri (originating in sacrum)

    Energy Technology Data Exchange (ETDEWEB)

    Resnik, C.S.; Young, J.W.R. (Maryland Univ., Baltimore, MD (USA). Dept. of Diagnostic Radiology); Levine, A.M. (Maryland Univ., Baltimore, MD (USA). Div. of Orthopaedic Surgery); Aisner, S.C. (Maryland Univ., Baltimore (USA). Dept. of Pathology)

    1989-07-01

    An elderly woman develops metastases to both humeri from a sacral chordoma leading to pathological fractures. This represents only the second reported case of such metastasis to a long bone from this primary site. The incidence of skeletal metastasis from chordoma was considered and the literature was reviewed. (orig.).

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

    International Nuclear Information System (INIS)

    Baek, Seung Hwan; Yu, In Kyu; Kim, Seong Min; Park, Ki Seok; Son, Hyun Jin

    2015-01-01

    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

  1. 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.

  2. 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

  3. A case report of craniovertebral junction intradural extramedullary neurenteric cyst

    Directory of Open Access Journals (Sweden)

    Rajeshwari S Vhora

    2014-01-01

    Full Text Available A neurenteric cyst of the craniocervical (CV junction, as a cause of bulbomedullary compression, is very rare. An abnormal communication between the endoderm and neuroectoderm during the third week of embryogenesis may be responsible for its formation. It is a rare spinal condition. The most frequent location is at the lower cervical and higher thoracic spine. Neurenteric cysts of the craniocervical junction are even rarer. We report the case of a CV junction intradural neurenteric cyst. Magnetic Resonance Imaging (MRI of our patient demonstrated an intradural extramedullary process of the craniocervical junction. A surgical posterior approach allowed gross total resection of the lesion. The histopathology of the surgical specimen showed that the cyst wall was made up of fibrocollagen walls lined with a partially ciliated columnar epithelium.

  4. CT and MR imaging of high cervical intradural lipomas

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Joo Hyeong; Choi, Woo Suk; Lee, Sun Wha; Lim, Jae Hoon; Leem, Woon; Kim, Gook Ki; Rhee, Bong Arm [Kyung Hee University Hospital, Seoul (Korea, Republic of)

    1988-04-15

    Intradural spinal lipoma occurs in less than 1% of all spinal cord tumors. It has been described at every level of the spinal canal, although its most common location is the cervicothoracic and thoracic region. However, lipoma located in the high cervical region is very unusual. We described two cases, a teenager and an adult, with progressive neurologic deficit from such a lipomatous tumor, which were evaluated by CT scanning and MR imaging.

  5. Chordoma of skull base presenting as nasopharyngeal mass

    Directory of Open Access Journals (Sweden)

    Sant Prakash Kataria

    2013-01-01

    Full Text Available While the nasopharynx is most commonly regarded by the otolaryngologist as a primary site of neoplastic involvement, it is also an avenue of spread of base-of-the-skull tumors presenting as bulging nasopharyngeal masses. Chordoma is a relatively rare tumor of the skull base and sacrum thought to originate from embryonic remnants of the notochord. Chordomas arising from the skull base/clivus are typically locally aggressive with lytic bone destruction. The optimal treatment may be photon/proton radiotherapy alone or combined with a gross total resection, when feasible. We report a case of intracranial chordoma presenting as nasopharyngeal mass.

  6. 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 ...

  7. Primary sacrococcygeal chordoma with unusual skeletal muscle metastasis

    Directory of Open Access Journals (Sweden)

    Lisa Vu, MD

    2014-01-01

    Full Text Available Chordomas are rare neoplasms that do not often metastasize. Of the small percent that do metastasize, they very infrequently involve skeletal muscle. Only a few cases of skeletal muscle metastases have been reported in the literature. We report an unusual case of a patient with a primary sacrococcygeal chordoma who experienced a long period of remission but who subsequently developed recurrence and multiple metastatic lesions to skeletal muscles including the deltoid, triceps, and pectineus.

  8. Humeral metastasis from a sacrococcygeal chordoma: a case report

    Directory of Open Access Journals (Sweden)

    Sepidbakht Sepideh

    2011-08-01

    Full Text Available Abstract Introduction Chordomas are rare tumors of the skeletal system that arise from an intra-osseous benign precursor of notochordal cells. They are mainly locally aggressive. However, metastases to other sites, including the humeri, resulting in pathological fractures have been reported. We report the case of a patient with a metastatic chordoma that produced a pathologic fracture of the humerus. Case presentation We report the case of a 60-year-old Iranian woman who presented with a fracture of her right humerus following a minor trauma. She had a history of a sacrococcygeal chordoma. Histological and immunohistochemical studies of the fracture site suggested the diagnosis of a chordoma. Conclusions Chordoma is a rare tumor and rarely metastasizes, but it should be considered in the differential diagnosis of epithelioid bone tumors. The only current effective treatment for this type of tumor is carbon ion therapy. There is currently no effective medical therapy available for advanced chordoma, and this type of tumor is not very responsive to radiotherapy.

  9. Differential proteomic profiling of primary and recurrent chordomas.

    Science.gov (United States)

    Chen, Su; Xu, Wei; Jiao, Jian; Jiang, Dongjie; Liu, Jian; Chen, Tenghui; Wan, Zongmiao; Xu, Leqin; Zhou, Zhenhua; Xiao, Jianru

    2015-05-01

    Chordomas are locally destructive tumors with high rates of recurrence and a poor prognosis. The mechanisms involved in chordoma recurrence remain largely unknown. In the present study, we examined the proteomic profile of a chordoma primary tumor (CSO) and a recurrent tumor (CSR) through mass spectrum in a chordoma patient who underwent surgery. Bioinformatic analysis of the profile showed that 359 proteins had a significant expression difference and 21 pathways had a striking alteration between the CSO and the CSR. The CSR showed a significant increase in carbohydrate metabolism. Immunohistochemistry (IHC) confirmed that the cancer stem cell marker activated leukocyte cell adhesion molecule (ALCAM or CD166) expression level was higher in the recurrent than that in the primary tumor. The present study analyzed the proteomic profile change between CSO and CSR and identified a new biomarker ALCAM in recurrent chordomas. This finding sheds light on unraveling the pathophysiology of chordoma recurrence and on exploring more effective prognostic biomarkers and targeted therapies against this devastating disease.

  10. Carbon-11-methionine positron emission tomography imaging of chordoma

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, Hong [Department of Medical Imaging, National Institute of Radiological Sciences, Chiba (Japan); Department of Medical Imaging, Research Center Hospital for Charged Particle Therapy, National Institute of Radiological Sciences, 4-9-1, Anagawa, Inage-ku, 263-8555, Chiba (Japan); Yoshikawa, Kyosan; Tamura, Katsumi; Sagou, Kenji; Kandatsu, Susumu [Clinical Diagnosis Section, National Institute of Radiological Sciences, Chiba (Japan); Tian, Mei; Suhara, Tetsuya; Suzuki, Kazutoshi; Tanada, Shuji [Department of Medical Imaging, National Institute of Radiological Sciences, Chiba (Japan); Tsujii, Hirohiko [Research Center for Charged Particle Therapy, National Institute of Radiological Sciences, Chiba (Japan)

    2004-09-01

    Chordoma is a rare malignant bone tumor that arises from notochord remnants. This is the first trial to investigate the utility of {sup 11}C-methionine (MET) positron emission tomography (PET) in the imaging of chordoma before and after carbon-ion radiotherapy (CIRT). Fifteen patients with chordoma were investigated with MET-PET before and after CIRT and the findings analyzed visually and quantitatively. Tumor MET uptake was evaluated by tumor-to-nontumor ratio (T/N ratio). In 12 (80%) patients chordoma was clearly visible in the baseline MET-PET study with a mean T/N ratio of 3.3{+-}1.7. The MET uptake decreased significantly to 2.3{+-}1.4 after CIRT (P<0.05). A significant reduction in tumor MET uptake of 24% was observed after CIRT. Fourteen (93%) patients showed no local recurrence after CIRT with a median follow-up time of 20 months. This study has demonstrated that MET-PET is feasible for imaging of chordoma. MET-PET could provide important tumor metabolic information for the therapeutic monitoring of chordoma after CIRT. (orig.)

  11. Carbon-11-methionine positron emission tomography imaging of chordoma

    International Nuclear Information System (INIS)

    Zhang, Hong; Yoshikawa, Kyosan; Tamura, Katsumi; Sagou, Kenji; Kandatsu, Susumu; Tian, Mei; Suhara, Tetsuya; Suzuki, Kazutoshi; Tanada, Shuji; Tsujii, Hirohiko

    2004-01-01

    Chordoma is a rare malignant bone tumor that arises from notochord remnants. This is the first trial to investigate the utility of 11 C-methionine (MET) positron emission tomography (PET) in the imaging of chordoma before and after carbon-ion radiotherapy (CIRT). Fifteen patients with chordoma were investigated with MET-PET before and after CIRT and the findings analyzed visually and quantitatively. Tumor MET uptake was evaluated by tumor-to-nontumor ratio (T/N ratio). In 12 (80%) patients chordoma was clearly visible in the baseline MET-PET study with a mean T/N ratio of 3.3±1.7. The MET uptake decreased significantly to 2.3±1.4 after CIRT (P<0.05). A significant reduction in tumor MET uptake of 24% was observed after CIRT. Fourteen (93%) patients showed no local recurrence after CIRT with a median follow-up time of 20 months. This study has demonstrated that MET-PET is feasible for imaging of chordoma. MET-PET could provide important tumor metabolic information for the therapeutic monitoring of chordoma after CIRT. (orig.)

  12. Primary intradural mesenchymal chondrosarcoma of the spine in a child

    Energy Technology Data Exchange (ETDEWEB)

    Li, Yu-Hua [Shanghai Jiao Tong University, Department of Radiology, Xin Hua Hospital, School of Medicine, Shanghai (China); Yao, Xiao-Hong [Shanghai Jiao Tong University, Department of Pathology, Xin Hua Hospital, School of Medicine, Shanghai (China)

    2007-11-15

    We report a primary intradural mesenchymal chondrosarcoma of the spine in a 3-year-old girl. MRI revealed a markedly enhancing oval mass associated with focal areas of low signal intensity extending from T11 to L1. The lesion was located posterolateral to the right side of the spinal cord, pushing the conus medullaris and cauda equina anteriorly and to the left. The adjacent spinal cord also showed serpiginous areas of flow void. The mass was completely removed. Microscopic examination and immunohistochemical studies confirmed the diagnosis of mesenchymal chondrosarcoma. The patient was free of symptoms after surgery. (orig.)

  13. Primary intradural mesenchymal chondrosarcoma of the spine in a child

    International Nuclear Information System (INIS)

    Li, Yu-Hua; Yao, Xiao-Hong

    2007-01-01

    We report a primary intradural mesenchymal chondrosarcoma of the spine in a 3-year-old girl. MRI revealed a markedly enhancing oval mass associated with focal areas of low signal intensity extending from T11 to L1. The lesion was located posterolateral to the right side of the spinal cord, pushing the conus medullaris and cauda equina anteriorly and to the left. The adjacent spinal cord also showed serpiginous areas of flow void. The mass was completely removed. Microscopic examination and immunohistochemical studies confirmed the diagnosis of mesenchymal chondrosarcoma. The patient was free of symptoms after surgery. (orig.)

  14. Metastatic carcinoma of breast or a chordoma? A case report and clinical perspectives.

    Science.gov (United States)

    Trivedi, Sachin; Odrazka, Karel

    2015-01-01

    We present a case of chordoma in a patient who had been previously treated for ductal carcinoma of the breast. The initial clinical findings and radiological studies suggested a possibility of metastases. However, the findings also adhered to the classical presentations and findings of the chordoma of the base of skull. It was only after the surgical resection and immunohistochemical confirmation that the diagnosis of chordoma could be established. Here, we discuss chordoma with the analysis of our clinical intrigue.

  15. Petrous apex chordoma - a case report; Cordoma de apice petroso - relato de um caso

    Energy Technology Data Exchange (ETDEWEB)

    Silveira, Claudio Regis S. [Clinica Boghos Boyadjian, Fortaleza, CE (Brazil); Barreto, Cristina Marques; Rossi, Luis Antonio [Hospital do Servidor Publico Estadual de Sao Paulo, SP (Brazil). Servico de Radiologia; Michiloski, Custodio; Rotta, Jose Marcus [Hospital do Servidor Publico Estadual de Sao Paulo, SP (Brazil). Servico de Neurocirurgia; Almeida, Serguey Malaquias de

    2001-02-01

    Chordomas are rare neoplasms arising from notochordal remnants that persist along the axial skeleton. Intracranial chordomas occur more frequently in the midline. We describe a typical 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. (author)

  16. Sacrococcygeal chordomas with wide-spread metastases: report of two cases and review of literature

    Energy Technology Data Exchange (ETDEWEB)

    Suh, Hyun Suk; Shin, Young Ju; Joo, Mee; Kim, Byung Jik [College of Medicine, Inje Univ., Pusan (Korea, Republic of)

    1999-03-01

    Chordomas are rare tumors arising from the primitive notochord. The commonest affected segment is the sacrum and these chordomas frequently follow a progressive course with multiple recurrences and metastases and eventual death due to tumor. This report describes two cases of sacrococcygeal chordomas with widespread metastases treated by surgery and adjuvant radiation therapy.

  17. Sacrococcygeal chordomas with wide-spread metastases: report of two cases and review of literature

    International Nuclear Information System (INIS)

    Suh, Hyun Suk; Shin, Young Ju; Joo, Mee; Kim, Byung Jik

    1999-01-01

    Chordomas are rare tumors arising from the primitive notochord. The commonest affected segment is the sacrum and these chordomas frequently follow a progressive course with multiple recurrences and metastases and eventual death due to tumor. This report describes two cases of sacrococcygeal chordomas with widespread metastases treated by surgery and adjuvant radiation therapy

  18. 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.

  19. Evaluation of intradural stimulation efficiency and selectivity in a computational model of spinal cord stimulation.

    Directory of Open Access Journals (Sweden)

    Bryan Howell

    Full Text Available Spinal cord stimulation (SCS is an alternative or adjunct therapy to treat chronic pain, a prevalent and clinically challenging condition. Although SCS has substantial clinical success, the therapy is still prone to failures, including lead breakage, lead migration, and poor pain relief. The goal of this study was to develop a computational model of SCS and use the model to compare activation of neural elements during intradural and extradural electrode placement. We constructed five patient-specific models of SCS. Stimulation thresholds predicted by the model were compared to stimulation thresholds measured intraoperatively, and we used these models to quantify the efficiency and selectivity of intradural and extradural SCS. Intradural placement dramatically increased stimulation efficiency and reduced the power required to stimulate the dorsal columns by more than 90%. Intradural placement also increased selectivity, allowing activation of a greater proportion of dorsal column fibers before spread of activation to dorsal root fibers, as well as more selective activation of individual dermatomes at different lateral deviations from the midline. Further, the results suggest that current electrode designs used for extradural SCS are not optimal for intradural SCS, and a novel azimuthal tripolar design increased stimulation selectivity, even beyond that achieved with an intradural paddle array. Increased stimulation efficiency is expected to increase the battery life of implantable pulse generators, increase the recharge interval of rechargeable implantable pulse generators, and potentially reduce stimulator volume. The greater selectivity of intradural stimulation may improve the success rate of SCS by mitigating the sensitivity of pain relief to malpositioning of the electrode. The outcome of this effort is a better quantitative understanding of how intradural electrode placement can potentially increase the selectivity and efficiency of SCS

  20. Genomic and epigenetic instability in chordoma: current insights

    Directory of Open Access Journals (Sweden)

    Feng Y

    2014-05-01

    Full Text Available Yong Feng,1,2 Jacson K Shen,1,3 Francis J Hornicek,1,3 Zhenfeng Duan1,3 1Department of Orthopedic Surgery, Massachusetts General Hospital, Boston, MA, USA; 2Department of Orthopedic Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, People’s Republic of China; 3Sarcoma Biology Laboratory, Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital, Boston, MA, USA Abstract: Chordoma is a malignant bone tumor, which currently can only be defined by histologic and immunohistochemical criteria. There are no prognostic biomarkers to predict the clinical outcome or response to treatment yet. Currently, chordoma pathogenesis is very poorly understood; however, recent large-scale genetic and epigenetic studies have identified some of the underlying mechanisms and pathways that may contribute to the disease. In this review, we summarize the most recent findings in the field of chordoma genomics and epigenomics, from comparative genomic hybridization to evaluate chromosomal alteration, large-scale deoxyribonucleic acid (DNA sequencing to determine the gene mutation, microarray to access messenger ribonucleic acid (RNA and microRNA gene expression, and DNA-methylation profiling. These studies may also hold valuable clinical potential in the management of chordoma. Keywords: chordoma, chromosomal alterations, sequencing, miRNA, DNA methylation

  1. Primary chondroid chordoma arising from the petrous temporal bone: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Young Uk; Youn, Eun Kyung [Koryo General Hospital, Seoul (Korea, Republic of)

    1991-01-15

    Chordomas are uncommon tumors which arise from remnants of the primitive notochord. They are situated chiefly in the anterior spinal axis with a predilection for the sacrococcygeal region and the basiocciput. About 50% of chordomas are sacrococcygeal, 35% are intracranial, and 15% arise from a vertebral body. As a histologic variant of chordoma, /chondroid chordoma' was first described by Heffelfinger et al. We present a rare case of primary chondroid chordoma arising from the petrous temporal bone. To our knowledge, only two other cases of this type have been reported earlier.

  2. Primary chondroid chordoma arising from the petrous temporal bone: a case report

    International Nuclear Information System (INIS)

    Lee, Young Uk; Youn, Eun Kyung

    1991-01-01

    Chordomas are uncommon tumors which arise from remnants of the primitive notochord. They are situated chiefly in the anterior spinal axis with a predilection for the sacrococcygeal region and the basiocciput. About 50% of chordomas are sacrococcygeal, 35% are intracranial, and 15% arise from a vertebral body. As a histologic variant of chordoma, /chondroid chordoma' was first described by Heffelfinger et al. We present a rare case of primary chondroid chordoma arising from the petrous temporal bone. To our knowledge, only two other cases of this type have been reported earlier

  3. Dorsal approaches to intradural extramedullary tumors of the craniovertebral junction

    Directory of Open Access Journals (Sweden)

    D Refai

    2010-01-01

    Full Text Available Tumors of the craniovertebral junction (CVJ pose significant challenges to cranial and spine surgeons. Familiarity with the complex anatomy and avoidance of injury to neurologic and vascular structures are essential to success. Multiple surgical approaches to address lesions at the CVJ have been promoted, including ventral and dorsal-based trajectories. However, optimal selection of the surgical vector to manage the pathology requires a firm understanding of the limitations and advantages of each approach. The selection of the best surgical trajectory must include several factors, such as obtaining the optimal exposure of the region of interest, avoiding injury to critical neurologic or vascular structures, identification of normal anatomical landmarks, the familiarity and comfort level of the surgeon to the approach, and the need for fixation. This review article focuses on dorsal approaches to the CVJ and the advantages and limitations in managing intradural extramedullary tumors.

  4. Extramedullary intradural spinal tumors; Extramedullaere intradurale spinale Tumoren

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    Papanagiotou, P. [Universitaetsklinikum des Saarlandes, Klinik fuer Diagnostische und Interventionelle Neuroradiologie, Homburg/Saar (Germany)

    2011-12-15

    The category of extramedullary intradural tumors includes a variety of lesions ranging from meningiomas originating from meningeal cells and nerve sheath tumors (neurofibromas, schwannomas) to less common primary tumors, such as lipomas, ependymomas, hemangiopericytomas, epidermoid cysts and dermoid cysts. Extramedullary metastases can occur as transcoelomic metastases in tumors of the central nervous system (CNS) or metastasization from other tumors. Magnetic resonance imaging (MRI) is the method of choice for localization and characterization of these lesions before treatment. (orig.) [German] Die Kategorie der extramedullaeren intraduralen Tumoren enthaelt Laesionen, die von den Nervenhuellen (Schwannome und Neurofibrome) oder von den meningealen Zellen ausgehen (Meningeome). Ependymome, Lipome, Haemangioperizytome, Epidermoidzysten und Dermoidzysten entsprechen selteneren primaeren Tumoren. Extramedullaere Metastasen koennen als Abtropfmetastasen bei ZNS-Tumoren oder als Metastasierung anderer Karzinomerkrankungen auftreten. Die Magnetresonanztomographie (MRT) ist die Methode der Wahl zur Abklaerung einer intraduralen Raumforderung. (orig.)

  5. Endoscopic Resection of Clival Chordoma. A Case Report

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    Ines Gamboa

    2018-01-01

    Full Text Available Introduction: Chordoma is a rare malignant tumor that arises from remnants cells of the primitive notochord, which are located at caudal and cephalic ends of the vertebral column. It represents 2 to 5 % of all primary bone tumors. Description: We report the case of a patient with a clival chordoma, asymptomatic, diagnosed as an accidental finding in a paranasal sinus. imaging study. Discussion: The imaging findings were suggestive of a potentially malignant lesion given the underlying bone lysis. Once the diagnosis is histological, biopsy of clival suspicious lesions should be promptly carried out. In this case report, the surgical approach and the postoperative follow-up are presented.

  6. Magnetic resonance (MR) imaging of chordoma and chondroma in the skull base

    International Nuclear Information System (INIS)

    Tashiro, Takahiko; Inoue, Yuichi; Nemoto, Yutaka

    1992-01-01

    Differential diagnosis of chordoma and chondroma in the skull base is sometimes difficult. We retrospectively reviewed the MR images of 14 patients with skull base tumors (nine chordomas, four chondromas and one chondrosarcoma). MR imaging was performed with a 0.5 Tesla system (Picker International). Inversion recovery (IR) (2500-2100/600-500/40), T1-weighted spin echo (SE) (800-600/40), and T2-weighted SE (2500-1800/120) images were obtained. On IR images, seven of eight chordomas showed heterogeneous low signal intensity, and one chordoma and all chondromas showed markedly low signal intensity similar to that of CSF. Calcified or ossified portions of the chondromas were demonstrated as areas of moderately low intensity on IR images. Chondrosarcoma showed moderately low intensity similar to that of chordoma. T1-weighted SE images of chordoma and chondroma showed no difference in signal intensity. On T2-weighted SE images, six of nine chordomas and all chondromas showed markedly high signal intensity. Three chordomas and one chondrosarcoma showed moderately high signal intensity. In the diagnosis of skull base tumors, the IR sequence seems to be useful for differentiating chondroma from chordoma. (author)

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

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    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.

  8. Thoracic intradural arachnoid cyst: Possible pitfalls with myelo-CT and MR

    International Nuclear Information System (INIS)

    Dietemann, J.L.; Filippi de la Palavesa, M.M.; Kastler, B.; Warter, J.M.; Buchheit, F.

    1991-01-01

    A thoracic intradural arachnoid cyst presenting as an intradural extramedullary mass highly suggestive of psammoma on myelogram and myelo-CT is reported in a 34-year-old female. High densities of the cyst were related to collection of contrast media within the cyst. However MR examination of the thoracic spinal cord including sagittal T1 (without and with contrast) and T2 studies failed to demonstrate the mass. Lack of MR changes were related on one hand to the small size of the cyst and to the absence of mass effect on the spinal cord, and on the other hand to a CSF-like signal of the contents of the cyst. Only combination of myelography, myelo-CT and MR allows precise diagnosis of small intradual arachnoid cysts; however MR is the method of choice for evaluation of large intradural subarachnoid cysts. (orig.)

  9. Analysis of radiological features relative to histopathology in 42 skull-base chordomas and chondrosarcomas

    International Nuclear Information System (INIS)

    Pamir, M. Necmettin; Ozduman, Koray

    2006-01-01

    Chordomas and chondrosarcomas are malignant tumors that are reported to have similar clinical presentations and radiological features but different behaviors and outcomes. The aim of this retrospective study was to determine whether specific radiological features of skull-base chordomas or chondrosarcomas are correlated with histopathology, and thus allow preoperative diagnosis. The study involved 32 classic chordomas, 6 chondroid chordomas and 4 chondrosarcomas (42 tumors total). For each case, tumor size and extent, the detailed anatomy involved, and magnetic resonance imaging and computed tomography findings were analyzed. Tumor extent was assessed using a novel method that assessed presence/absence in 18 defined skull-base zones. The chondrosarcomas presented significantly earlier in life than the chordomas (means, 20.5 years versus 36 years, respectively). At time of diagnosis, the median tumor volume was 23 cm 3 (range, 1.2-78.8 cm 3 ) and the mean tumor extent was 6.7 ± 2.9 zones. There were no differences between chordomas and chondrosarcomas, or between the two chordoma subgroups, with respect to lesion volume or extent. Comparison of other imaging findings revealed no features that were diagnostic for either chordoma or chondrosarcoma. The data support previous claims that chondrosarcomas present earlier in life than chordomas, but this finding is not diagnostic. There is wide variation in the extent of skull-base chordomas and chondrosarcomas, and in the specific anatomical structures these tumors involve. None of the MRI or CT features of these tumors appear to be useful for differentiating chordomas from chondrosarcomas preoperatively. For surgical planning, specific, area-oriented definition of tumor extent might provide more useful information than tumor-type classification schemes

  10. Analysis of radiological features relative to histopathology in 42 skull-base chordomas and chondrosarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Pamir, M. Necmettin [Marmara University Faculty of Medicine, Department of Neurosurgery, Istanbul (Turkey)]. E-mail: koray.ozduman@yale.edu; Ozduman, Koray [Marmara University Faculty of Medicine, Department of Neurosurgery, Istanbul (Turkey)

    2006-06-15

    Chordomas and chondrosarcomas are malignant tumors that are reported to have similar clinical presentations and radiological features but different behaviors and outcomes. The aim of this retrospective study was to determine whether specific radiological features of skull-base chordomas or chondrosarcomas are correlated with histopathology, and thus allow preoperative diagnosis. The study involved 32 classic chordomas, 6 chondroid chordomas and 4 chondrosarcomas (42 tumors total). For each case, tumor size and extent, the detailed anatomy involved, and magnetic resonance imaging and computed tomography findings were analyzed. Tumor extent was assessed using a novel method that assessed presence/absence in 18 defined skull-base zones. The chondrosarcomas presented significantly earlier in life than the chordomas (means, 20.5 years versus 36 years, respectively). At time of diagnosis, the median tumor volume was 23 cm{sup 3} (range, 1.2-78.8 cm{sup 3}) and the mean tumor extent was 6.7 {+-} 2.9 zones. There were no differences between chordomas and chondrosarcomas, or between the two chordoma subgroups, with respect to lesion volume or extent. Comparison of other imaging findings revealed no features that were diagnostic for either chordoma or chondrosarcoma. The data support previous claims that chondrosarcomas present earlier in life than chordomas, but this finding is not diagnostic. There is wide variation in the extent of skull-base chordomas and chondrosarcomas, and in the specific anatomical structures these tumors involve. None of the MRI or CT features of these tumors appear to be useful for differentiating chordomas from chondrosarcomas preoperatively. For surgical planning, specific, area-oriented definition of tumor extent might provide more useful information than tumor-type classification schemes.

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

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    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.

  12. 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).

  13. Imaging features of posterior mediastinal chordoma in a child

    Energy Technology Data Exchange (ETDEWEB)

    Soudack, Michalle; Guralnik, Ludmilla; Engel, Ahuva [Rambam Health Care Campus, Department of Diagnostic Imaging, Haifa (Israel); Ben-Nun, Alon [Rambam Health Care Campus, Department of Thoracic Surgery, Haifa (Israel); Berkowitz, Drora [Rambam Health Care Campus, Department of Pediatrics B, Haifa (Israel); Postovsky, Sergey [Rambam Health Care Campus, Department of Pediatric Hemato-Oncology, Haifa (Israel); Vlodavsky, Eugene [Rambam Health Care Campus, Department of Pathology, Haifa (Israel)

    2007-05-15

    A 51/2-year-old boy presented with repeated episodes of stridor and cough. Chest radiography demonstrated a widened mediastinum. Evaluation by CT revealed a low-density posterior mediastinal mass initially diagnosed as benign tumor. Histopathological analysis of the resected mass disclosed a malignant chordoma. Our radiological results are described with an analysis of the imaging findings in the medical literature. We present our suggestions for preoperative evaluation of posterior mediastinal tumors. (orig.)

  14. Computer Navigation-aided Resection of Sacral Chordomas

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    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.

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

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    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.

  16. MRI with gadolinium DTPA in the diagnosis of spinal intradural masses

    International Nuclear Information System (INIS)

    Kahn, T.; Fuerst, G.; Moedder, U.; Roosen, N.; Lins, E.; Bock, W.J.; Lenard, H.G.

    1989-01-01

    The results of contrast enhanced MRI in 36 patients with suspected spinal intradural tumours are described. All intramedullary tumours showed distinctive enhancement and solid tumors could be delineated clearly, even if they were not clearly visible on unenhanced scans. The differentiation between neoplasm and non-neoplastic syrinx was markedly improved. The sensitivity of MRI for demonstrating intradural extramedullary tumours was greatly improved by gadolinium DTPA and even small lesions or flat meningeal infiltrates could be visualised. In addition, gadolinium DTPA improved the delineation and localisation of larger lesions, even if they had already been seen on unenhanced images. (orig.) [de

  17. On a Rare Cutaneous Metastasis from a Sacrococcygeal Chordoma

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    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.

  18. Characteristics and Patterns of Metastatic Disease from Chordoma

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    Victoria A. Young

    2015-01-01

    Full Text Available Chordoma is a rare, slow-growing malignant tumor arising from notochordal remnants. A retrospective review of patient records at two major referral centers was undertaken to assess the incidence, location, and prognostic factors of metastatic disease from chordoma. 219 patients with chordoma (1962–2009 were identified. 39 patients (17.8% developed metastatic disease, most frequently to lung (>50%. Median survival from the time of initial diagnosis was 130.4 months for patients who developed metastatic disease and 159.3 months for those who did not (P=0.05. Metastatic disease was most common in the youngest patients (P=0.07, and it was 2.5 times more frequent among patients with local recurrence (26.3% than in those without (10.8% (P=0.003. Patient survival with metastatic disease was highly variable, and it was dependent on both the location of the tumor primary and the site of metastasis. Metastasis to distal bone was the most rapid to develop and had the worst prognosis.

  19. Clinical results of proton beam therapy for skull base chordoma

    International Nuclear Information System (INIS)

    Igaki, Hiroshi; Tokuuye, Koichi; Okumura, Toshiyuki; Sugahara, Shinji; Kagei, Kenji; Hata, Masaharu; Ohara, Kiyoshi; Hashimoto, Takayuki; Tsuboi, Koji; Takano, Shingo; Matsumura, Akira; Akine, Yasuyuki

    2004-01-01

    Purpose: To evaluate clinical results of proton beam therapy for patients with skull base chordoma. Methods and materials: Thirteen patients with skull base chordoma who were treated with proton beams with or without X-rays at the University of Tsukuba between 1989 and 2000 were retrospectively reviewed. A median total tumor dose of 72.0 Gy (range, 63.0-95.0 Gy) was delivered. The patients were followed for a median period of 69.3 months (range, 14.6-123.4 months). Results: The 5-year local control rate was 46.0%. Cause-specific, overall, and disease-free survival rates at 5 years were 72.2%, 66.7%, and 42.2%, respectively. The local control rate was higher, without statistical significance, for those with preoperative tumors <30 mL. Partial or subtotal tumor removal did not yield better local control rates than for patients who underwent biopsy only as the latest surgery. Conclusion: Proton beam therapy is effective for patients with skull base chordoma, especially for those with small tumors. For a patient with a tumor of <30 mL with no prior treatment, biopsy without tumor removal seems to be appropriate before proton beam therapy

  20. 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

  1. High-resolution Whole-Genome Analysis of Skull Base Chordomas Implicates FHIT Loss in Chordoma Pathogenesis12

    OpenAIRE

    Diaz, Roberto Jose; Guduk, Mustafa; Romagnuolo, Rocco; Smith, Christian A; Northcott, Paul; Shih, David; Berisha, Fitim; Flanagan, Adrienne; Munoz, David G; Cusimano, Michael D; Pamir, M Necmettin; Rutka, James T

    2012-01-01

    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 ...

  2. The importance of preoperative tissue sampling for mobile spine chordomas: literature review and report of two cases.

    Science.gov (United States)

    Zuccato, Jeffrey A; Witiw, Christopher D; Keith, Julia; Dyer, Erin; Saghal, Arjun; da Costa, Leodante

    2018-01-01

    Pre-operative biopsy and diagnosis of chordomas of the mobile spine is indicated as en bloc resections improve outcomes. This review of the management of mobile spine chordomas includes two cases of unexpected mobile spine chordomas where a preoperative tissue diagnosis was decided against and may have altered surgical decision-making. Two lumbar spine chordomas thought to be metastatic and primary bony lesions preoperatively were not biopsied before surgery and eventual pathology revealed chordoma. Preoperative diagnoses were questioned during surgery after an intraoperative tissue diagnosis of chordoma in one case and unclear pathology with non-characteristic tumor morphology in the other. The surgical plan was altered in these cases to maximize resection as en bloc resection reduces the risk of local recurrence in chordoma. Mobile spine chordomas are rare and en bloc resection is recommended, contrary to the usual approach to more common spine tumors. Since en bloc resection of spine chordomas improves disease free survival, it has been recommended that tissue diagnosis be obtained preoperatively when chordoma is considered in the differential diagnosis, in order to guide surgical planning. We present two cases where a preoperative biopsy was considered but not obtained after neuroradiology consultation and imaging review, which may have been managed differently if the diagnosis of spine chordomas were known pre-operatively.

  3. Management of advanced intracranial intradural juvenile nasopharyngeal angiofibroma: combined single-stage rhinosurgical and neurosurgical approach.

    Science.gov (United States)

    Naraghi, Mohsen; Saberi, Hooshang; Mirmohseni, Atefeh Sadat; Nikdad, Mohammad Sadegh; Afarideh, Mohsen

    2015-07-01

    Although intracranial extension of juvenile nasopharyngeal angiofibroma (JNA) occurs commonly, intradural penetration is extremely rare. Management of such tumors is a challenging issue in skull-base surgery, necessitating their removal via combined approaches. In this work, we share our experience in management of extensive intradural JNA. In a university hospital-based setting of 2 tertiary care academic centers, retrospective chart of 6 male patients (5 between 15 and 19 years old) was reviewed. Patients presented chiefly with nasal obstruction, epistaxis, and proptosis. One of them was an aggressive recurrent tumor in a 32-year-old patient. All cases underwent combined transnasal, transmaxillary, and craniotomy approaches assisted by the use of image-guided endoscopic surgery, with craniotomy preceding the rhinosurgical approach in 3 cases. Adding a transcranial approach to the transnasal and transmaxillary endoscopic approaches provided 2-sided exposure and appreciated access to the huge intradural JNAs. One postoperative cerebrospinal fluid leak and 1 postoperative recurrence at the site of infratemporal fossa were treated successfully. Otherwise, the course was uneventful in the remaining cases. Management of intracranial intradural JNA requires a multidisciplinary approach of combined open and endoscopic-assisted rhinosurgery and neurosurgery, because of greater risk for complications during the dissection. Carotid rupture and brain damage remain 2 catastrophic complications that should always be kept in mind. A combined rhinosurgical and neurosurgical approach also has the advantage of very modest cosmetic complications. © 2015 ARS-AAOA, LLC.

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

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    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.

  5. Gamma Knife surgery for intracranial chordoma and chondrosarcoma: radiosurgical perspectives and treatment outcomes.

    Science.gov (United States)

    Kim, Ji Hee; Jung, Hyun Ho; Chang, Jong Hee; Chang, Jin Woo; Park, Yong Gou; Chang, Won Seok

    2014-12-01

    Intracranial chordomas and chondrosarcomas are histologically low-grade, locally invasive tumors that are reported to be similar in terms of anatomical location, clinical presentation, and radiological findings but different in terms of behavior and outcomes. The purpose of this study was to investigate and compare clinical outcomes after Gamma Knife surgery (GKS) for the treatment of intracranial chordoma and chondrosarcoma. The authors conducted a retrospective review of the results of radiosurgical treatment of intracranial chordomas and chondrosarcomas. They enrolled patients who had undergone GKS for intracranial chordoma or chondrosarcoma at the Yonsei Gamma Knife Center, Yonsei University College of Medicine, from October 2000 through June 2007. Analyses included only patients for whom the disease was pathologically diagnosed before GKS and for whom more than 5 years of follow-up data after GKS were available. Rates of progression-free survival and overall survival were analyzed and compared according to tumor pathology. Moreover, the association between tumor control and the margin radiation dose to the tumor was analyzed, and the rate of tumor volume change after GKS was quantified. A total of 10 patients were enrolled in this study. Of these, 5 patients underwent a total of 8 sessions of GKS for chordoma, and the other 5 patients underwent a total of 7 sessions of GKS for chondrosarcoma. The 2- and 5-year progression-free survival rates for patients in the chordoma group were 70% and 35%, respectively, and rates for patients in the chondrosarcoma group were 100% and 80%, respectively (log-rank test, p = 0.04). The 2- and 5-year overall survival rates after GKS for patients in the chordoma group were 87.5% and 72.9%, respectively, and rates for patients in the chondrosarcoma group were 100% and 100%, respectively (log-rank test, p = 0.03). The mean rates of tumor volume change 2 years after radiosurgery were 79.64% and 39.91% for chordoma and

  6. 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

  7. Tissue microarray immunohistochemical detection of brachyury is not a prognostic indicator in chordoma.

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    Linlin Zhang

    Full Text Available Brachyury is a marker for notochord-derived tissues and neoplasms, such as chordoma. However, the prognostic relevance of brachyury expression in chordoma is still unknown. The improvement of tissue microarray technology has provided the opportunity to perform analyses of tumor tissues on a large scale in a uniform and consistent manner. This study was designed with the use of tissue microarray to determine the expression of brachyury. Brachyury expression in chordoma tissues from 78 chordoma patients was analyzed by immunohistochemical staining of tissue microarray. The clinicopathologic parameters, including gender, age, location of tumor and metastatic status were evaluated. Fifty-nine of 78 (75.64% tumors showed nuclear staining for brachyury, and among them, 29 tumors (49.15% showed 1+ (<30% positive cells staining, 15 tumors (25.42% had 2+ (31% to 60% positive cells staining, and 15 tumors (25.42% demonstrated 3+ (61% to 100% positive cells staining. Brachyury nuclear staining was detected more frequently in sacral chordomas than in chordomas of the mobile spine. However, there was no significant relationship between brachyury expression and other clinical variables. By Kaplan-Meier analysis, brachyury expression failed to produce any significant relationship with the overall survival rate. In conclusion, brachyury expression is not a prognostic indicator in chordoma.

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

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    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.

  9. Clival chordomas: considerations after 16 years of endoscopic endonasal surgery.

    Science.gov (United States)

    Zoli, Matteo; Milanese, Laura; Bonfatti, Rocco; Faustini-Fustini, Marco; Marucci, Gianluca; Tallini, Giovanni; Zenesini, Corrado; Sturiale, Carmelo; Frank, Giorgio; Pasquini, Ernesto; Mazzatenta, Diego

    2018-02-01

    OBJECTIVE In the past decade, the role of the endoscopic endonasal approach (EEA) has relevantly evolved for skull base tumors. In this study, the authors review their surgical experience with using an EEA in the treatment of clival chordomas, which are deep and infiltrative skull base lesions, and they highlight the advantages and limitations of this ventral approach. METHODS All consecutive cases of chordoma treated with an EEA between 1998 and 2015 at a single institution are included in this study. Preoperative assessment consisted of neuroimaging (MRI and CT with angiography sequences) and endocrinological, neurological, and ophthalmological evaluations, which were repeated 3 months after surgery and annually thereafter. Postoperative adjuvant therapies were considered. RESULTS Sixty-five patients (male/female ratio 1:0.9) were included in this study. The median age was 48 years (range 9-80 years). Gross-total resection (GTR) was achieved in 47 cases (58.7%). On univariate analysis, primary procedures (p = 0.001), location in the superior or middle third of the clivus (p = 0.043), extradural location (p = 0.035), and histology of conventional chordomas (p = 0.013) were associated with a higher rate of GTR. The complication rate was 15.1%, and there were no perioperative deaths. Most complications did not result in permanent sequelae and included 2 CSF leaks (2.5%), 5 transient cranial nerve VI palsies (6.2%), and 2 internal carotid artery injuries (2.5%), which were treated with coil occlusion of the internal carotid artery without neurological deficits. Three patients (3.8%) presented with complications resulting in permanent neurological deficits due to a postoperative hematoma (1.2%) causing a hemiparesis, and 2 permanent ophthalmoplegias (2.5%). Seventeen patients (26.2%) have died of tumor progression over the course of follow-up (median 52 months, range 7-159 months). Based on Kaplan-Meier analysis, the survival rate was 77% at 5 years and 57% at 10

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

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    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.)

  11. 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.)

  12. Cranial chordomas in infancy and childhood. A report of two cases and review of the literature

    International Nuclear Information System (INIS)

    Matsumoto, J.; Towbin, R.B.; Ball, W.S. Jr.; Children's Hospital Medical Center, Cincinnati, OH; Cincinnati Univ., OH

    1989-01-01

    Cranial chordomas are uncommon, accounting for less than 1% of all intracranial neoplasms. Although they are presumed to arise from congenital notochordal remnants, it is rare for these tumors to present in childhood. Only 35 cases of cranial chordomas have been reported in children 16 years of age or younger. We report 2 additional cases of pediatric cranial chordomas. One occurred in a 4 month old infant and to our knowledge represents the earliest age of presentation yet reported. The second case documents the value of MR imaging in delineating the extent of the tumor and defining its relationship to adjacent structures. (orig.)

  13. Chordoma: review of clinico radiological features and factors affecting survival

    International Nuclear Information System (INIS)

    Soo, M.Y.S.

    2001-01-01

    This study reviews the clinico radiological features of cranial and sacrospinal chordomas and identifies factors affecting survival. Nineteen patients seen between January 1980 and December 2000 with histopathological diagnosis of chordomas were retrospectively reviewed with reference to clinical presentation, imaging features, treatment modalities and post-therapy status. Eight had tumours in the skull base while 11 patients had spinal and sacro-coccygeal lesions. Surgical resection was performed in 16 patients whose subsequent natural history was used to identify clinical indicators that may influence survival. Completeness of resection, age, gender and postoperative irradiation were subjected to analysis using the Cox proportional hazard models. Kaplan-Meir survival curves illustrate the survival distributions. Diplopia and facial pain are prime clinical presentations in cranial lesions, while extremity weakness and a sacrogluteal mass are common complaints in the sacrospinal group. Lesional calcifications are present in 40% while an osteolytic soft tissue mass is detectable by CT in all cases. Heterogeneous signals and internal septations on T 2 -weighted MRI are predominant features. In sacrospinal tumours, complete excision with adjuvant radiotherapy achieves the best results with a disease-free survival of more than 5 years. The clinical and imaging findings in this study are in accordance with those of other series. Except for complete surgical excision followed by radiotherapy in the subset of patients with sacrospinal tumours, none of the other clinical indicators show a statistical significant influence on survival. Copyright (2001) Blackwell Science Pty Ltd

  14. 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)

  15. The Brachyury Gly177Asp SNP Is not Associated with a Risk of Skull Base Chordoma in the Chinese Population

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    Zhen Wu

    2013-10-01

    Full Text Available A recent chordoma cancer genotyping study reveals that the rs2305089, a single nucleotide polymorphism (SNP located in brachyury gene and a key gene in the development of notochord, is significantly associated with chordoma risk. The brachyury gene is believed to be one of the key genes involved in the pathogenesis of chordoma, a rare primary bone tumor originating along the spinal column or at the base of the skull. The association between the brachyury Gly177Asp single nucleotide polymorphism (SNP and the risk of skull base chordoma in Chinese populations is currently unknown. We investigated the genotype distribution of this SNP in 65 skull-base chordoma cases and 120 healthy subjects. Comparisons of the genotype distributions and allele frequencies did not reveal any significant difference between the groups. Our data suggest that the brachyury Gly177Asp SNP is not involved in the risks of skull-base chordoma, at least in the Chinese population.

  16. Hérnias intradural de discos lombares: relato de dois casos

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    Joel M. Guelmann

    1972-09-01

    Full Text Available São relatados dois casos de hérnia de disco lombar intradural. Os autores salientam a raridade desta ocorrência e fazem comentários sobre os fatores que podem determina-la. Ambos os pacientes foram operados, com extirpação total da hérnia. Houve completa recuperação dos pacientes.

  17. Correlation of magnetic resonance imaging findings of spinal intradural extramedullary schwannomas with pathologic findings

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    Kim, Yeo Ju; Park, In Suh; Yoon, Seung Hwan; Choi, Suk Jin; Kim, Youn Jeong; Kang, Young Hye; Lee, Ha Young; Kim, Woo Chul; Han, Jun Gu; Cho, Soon Gu [Inha University Hospital, Incheon (Korea, Republic of)

    2015-06-15

    To evaluate the magnetic resonance imaging (MRI) findings of spinal intradural extramedullary schwannomas with pathologic correlation and to determine whether these schwannomas share the imaging features of schwannomas in the peripheral nerves. The MRIs of 17 cases of pathologically proven spinal intradural extramedullary schwannomas were reviewed retrospectively, and cystic changes, enhancement, and intratumoral hemorrhage of the tumors were evaluated. Imaging features known to be common findings of schwannoma in the peripheral nerves, such as encapsulation, the target sign, the fascicular sign, and visualization of entering or exiting nerve rootlets, were also evaluated. The histopathology of the tumors was correlated with the MRI findings. Cystic changes were detected in 14 cases by MRI and in 16 cases by pathology. The most common pattern of enhancement was a thick peripheral septal pattern (70.59%). Intratumoral hemorrhage was detected in four cases on MRI, but in all cases on pathology. Encapsulation was observed in all cases. The fascicular sign was seen in only four cases, and thickening of an exiting rootlet was visualized in one case. None of the cases showed the target sign. Spinal intradural extramedullary schwannomas were typical encapsulated cystic tumors and had few imaging features of schwannomas in the peripheral nerves.

  18. From notochord formation to hereditary chordoma: the many roles of Brachyury.

    Science.gov (United States)

    Nibu, Yutaka; José-Edwards, Diana S; Di Gregorio, Anna

    2013-01-01

    Chordoma is a rare, but often malignant, bone cancer that preferentially affects the axial skeleton and the skull base. These tumors are both sporadic and hereditary and appear to occur more frequently after the fourth decade of life; however, modern technologies have increased the detection of pediatric chordomas. Chordomas originate from remnants of the notochord, the main embryonic axial structure that precedes the backbone, and share with notochord cells both histological features and the expression of characteristic genes. One such gene is Brachyury, which encodes for a sequence-specific transcription factor. Known for decades as a main regulator of notochord formation, Brachyury has recently gained interest as a biomarker and causative agent of chordoma, and therefore as a promising therapeutic target. Here, we review the main characteristics of chordoma, the molecular markers, and the clinical approaches currently available for the early detection and possible treatment of this cancer. In particular, we report on the current knowledge of the role of Brachyury and of its possible mechanisms of action in both notochord formation and chordoma etiogenesis.

  19. A zebrafish model of chordoma initiated by notochord-driven expression of HRASV12.

    Science.gov (United States)

    Burger, Alexa; Vasilyev, Aleksandr; Tomar, Ritu; Selig, Martin K; Nielsen, G Petur; Peterson, Randall T; Drummond, Iain A; Haber, Daniel A

    2014-07-01

    Chordoma is a malignant tumor thought to arise from remnants of the embryonic notochord, with its origin in the bones of the axial skeleton. Surgical resection is the standard treatment, usually in combination with radiation therapy, but neither chemotherapeutic nor targeted therapeutic approaches have demonstrated success. No animal model and only few chordoma cell lines are available for preclinical drug testing, and, although no druggable genetic drivers have been identified, activation of EGFR and downstream AKT-PI3K pathways have been described. Here, we report a zebrafish model of chordoma, based on stable transgene-driven expression of HRASV12 in notochord cells during development. Extensive intra-notochordal tumor formation is evident within days of transgene expression, ultimately leading to larval death. The zebrafish tumors share characteristics of human chordoma as demonstrated by immunohistochemistry and electron microscopy. The mTORC1 inhibitor rapamycin, which has some demonstrated activity in a chordoma cell line, delays the onset of tumor formation in our zebrafish model, and improves survival of tumor-bearing fish. Consequently, the HRASV12-driven zebrafish model of chordoma could enable high-throughput screening of potential therapeutic agents for the treatment of this refractory cancer. © 2014. Published by The Company of Biologists Ltd.

  20. A zebrafish model of chordoma initiated by notochord-driven expression of HRASV12

    Directory of Open Access Journals (Sweden)

    Alexa Burger

    2014-07-01

    Full Text Available Chordoma is a malignant tumor thought to arise from remnants of the embryonic notochord, with its origin in the bones of the axial skeleton. Surgical resection is the standard treatment, usually in combination with radiation therapy, but neither chemotherapeutic nor targeted therapeutic approaches have demonstrated success. No animal model and only few chordoma cell lines are available for preclinical drug testing, and, although no druggable genetic drivers have been identified, activation of EGFR and downstream AKT-PI3K pathways have been described. Here, we report a zebrafish model of chordoma, based on stable transgene-driven expression of HRASV12 in notochord cells during development. Extensive intra-notochordal tumor formation is evident within days of transgene expression, ultimately leading to larval death. The zebrafish tumors share characteristics of human chordoma as demonstrated by immunohistochemistry and electron microscopy. The mTORC1 inhibitor rapamycin, which has some demonstrated activity in a chordoma cell line, delays the onset of tumor formation in our zebrafish model, and improves survival of tumor-bearing fish. Consequently, the HRASV12-driven zebrafish model of chordoma could enable high-throughput screening of potential therapeutic agents for the treatment of this refractory cancer.

  1. Proton Therapy for Reirradiation of Progressive or Recurrent Chordoma

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    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

  2. Proton Therapy for Reirradiation of Progressive or Recurrent Chordoma

    International Nuclear Information System (INIS)

    McDonald, Mark W.; Linton, Okechuckwu R.; Shah, Mitesh V.

    2013-01-01

    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 3 (range, 0-701 cm 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 toxicities may

  3. 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

  4. Osseous metastases of chordoma: imaging and clinical findings

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    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.)

  5. Investigating microenvironmental regulation of human chordoma cell behaviour.

    Directory of Open Access Journals (Sweden)

    Priya Patel

    Full Text Available The tumour microenvironment is complex and composed of many different constituents, including matricellular proteins such as connective tissue growth factor (CCN2, and is characterized by gradients in oxygen levels. In various cancers, hypoxia and CCN2 promote stem and progenitor cell properties, and regulate the proliferation, migration and phenotype of cancer cells. Our study was aimed at investigating the effects of hypoxia and CCN2 on chordoma cells, using the human U-CH1 cell line. We demonstrate that under basal conditions, U-CH1 cells express multiple CCN family members including CCN1, CCN2, CCN3 and CCN5. Culture of U-CH1 cells in either hypoxia or in the presence of recombinant CCN2 peptide promoted progenitor cell-like characteristics specific to the notochordal tissue of origin. Specifically, hypoxia induced the most robust increase in progenitor-like characteristics in U-CH1 cells, including increased expression of the notochord-associated markers T, CD24, FOXA1, ACAN and CA12, increased cell growth and tumour-sphere formation, and a decrease in the percentage of vacuolated cells present in the heterogeneous population. Interestingly, the effects of recombinant CCN2 peptide on U-CH1 cells were more pronounced under normoxia than hypoxia, promoting increased expression of CCN1, CCN2, CCN3 and CCN5, the notochord-associated markers SOX5, SOX6, T, CD24, and FOXA1 as well as increased tumour-sphere formation. Overall, this study highlights the importance of multiple factors within the tumour microenvironment and how hypoxia and CCN2 may regulate human chordoma cell behaviour.

  6. Proton Therapy for Skull Base Chordomas: An Outcome Study from the University of Florida Proton Therapy Institute

    OpenAIRE

    Deraniyagala, Rohan L.; Yeung, Daniel; Mendenhall, William M.; Li, Zuofeng; Morris, Christopher G.; Mendenhall, Nancy P.; Okunieff, Paul; Malyapa, Robert S.

    2013-01-01

    Objectives Skull base chordoma is a rare, locally aggressive tumor located adjacent to critical structures. Gross total resection is difficult to achieve, and proton therapy has the conformal advantage of delivering a high postoperative dose to the tumor bed. We present our experience using proton therapy to treat 33 patients with skull base chordomas.

  7. Intradural extramedullary Ewing's sarcoma: A case report and review of the literature.

    Science.gov (United States)

    Paterakis, Konstantinos; Brotis, Alexandros; Tasiou, Anastasia; Kotoula, Vasiliki; Kapsalaki, Eftychia; Vlychou, Marianna

    Extra-skeletal Ewing's sarcomas are very rare lesions to the spine surgeon, with the intradural, extramedullary lesions being even rarer. Herein we present a patient with an intradural, extramedullary form of Ewing's sarcoma and review the relevant literature. The medical records, operative reports, radiographical studies and histological examinations of a single patient are retrospectively reviewed. A 31-year old male presented with back-pain, right-leg progressive paraparesis, and inability to walk. Both motor and sensory disturbances were revealed on the right leg at the clinical examination. Lumbar MRI showed two lesions. The first one was an intradural, extramedullary lesion at the L2-L3 level, while the second was smaller, located at the bottom of the dural sac. The patient underwent gross total resection of the L2-L3 lesion after a bilateral laminectomy. Histological examination was compatible with Ewing's sarcoma, and was verified by molecular analysis. No other extra-skeletal or skeletal lesion was found. A chemotherapy scheme was tailored to the patients' histological diagnosis. The patient presented with local recurrence and bone metastasis 2 years after his initial diagnosis. A second operation was performed and the follow up of the patient showed no disease progression 18 months after revision surgery. The spine surgeon should be aware of the existence of such rare entities, in order to timely fulfill the staging process and institute the proper therapy. The management of patients with extra-skeletal Ewing's sarcomas involves professionals as members of a multidisciplinary team, all of which should co-operate for the patient's optimal outcome. Copyright © 2016. Published by Elsevier Urban & Partner Sp. z o.o.

  8. Chordoma versus chondrosarcoma of the central skull base: MR and CT findings

    International Nuclear Information System (INIS)

    Choi, Guk Myeong; Han, Moon Hee; Chang, Kee Hyun; Kim, Hong Dae; Yeon, Kyung Mo; Kim, Sam Soo

    1998-01-01

    It is known that due to both their imaging and pathologic features, the accurate differentiation of chondrosarcoma from chordoma is difficult. Through an analysis of MR and CT finding, this study aims to determine the differential points between these two tumors. In 21 patients, CT and MR imaging studies of chordoma (n=12) and chondrosarcoma (n=9) at the base of the skull were retrospectively reviewed. Diagnosis had been established by histologic examination of surgically removed specimens. Eleven of the chordomas were subclassified as conventional and one as chondroid ; eight chondrosarcoma were conventional and one was myxoid. Four chordoma patients underwent CT and MR ; in six, only MR was in one, only CT was performed. All scans were retrospectively evaluated for the location (midline/off-midline), direction of extension, margin and shape, bony destruction and calcification, MR signal intensity and enhancement patterns of the tumors. Degree of calcification was graded from I to II. Although MR and CT findings were similar in both types of tumor, location and degree of calcification may be features which usefully distinguish chordoma from chondrosarcoma. (author). 17 refs., 2 tabs., 5 figs

  9. Radiotherapy for chordomas and low-grade chondrosarcomas of the skull base with carbon ions

    International Nuclear Information System (INIS)

    Schulz-Ertner, Daniela; Haberer, Thomas; Jaekel, Oliver; Thilmann, Christoph; Kraemer, Michael; Enghardt, Wolfgang; Kraft, Gerhard; Wannenmacher, Michael; Debus, Juergen

    2002-01-01

    Purpose: Compared to photon irradiation, carbon ions provide physical and biologic advantages that may be exploited in chordomas and chondrosarcomas. Methods and Materials: Between August 1998 and December 2000, 37 patients with chordomas (n=24) and chondrosarcomas (n=13) were treated with carbon ion radiotherapy within a Phase I/II trial. Tumor conformal application of carbon ion beams was realized by intensity-controlled raster scanning with pulse-to-pulse energy variation. Three-dimensional treatment planning included biologic plan optimization. The median tumor dose was 60 GyE (GyE Gy x relative biologic effectiveness). Results: The mean follow-up was 13 months. The local control rate after 1 and 2 years was 96% and 90%, respectively. We observed 2 recurrences outside the gross tumor volume in patients with chordomas. Progression-free survival was 100% for chondrosarcomas and 83% for chordomas at 2 years. Partial remission after carbon ion radiotherapy was observed in 6 patients. Treatment toxicity was mild. Conclusion: These are the first data demonstrating the clinical feasibility, safety, and effectiveness of scanning beam delivery of ion beams in patients with skull base tumors. The preliminary results in patients with skull base chordomas and low-grade chondrosarcomas are encouraging, although the follow-up was too short to draw definite conclusions concerning outcome. In the absence of major toxicity, dose escalation might be considered

  10. Tissue microarray immunohistochemical detection of brachyury is not a prognostic indicator in chordoma.

    Science.gov (United States)

    Zhang, Linlin; Guo, Shang; Schwab, Joseph H; Nielsen, G Petur; Choy, Edwin; Ye, Shunan; Zhang, Zhan; Mankin, Henry; Hornicek, Francis J; Duan, Zhenfeng

    2013-01-01

    Brachyury is a marker for notochord-derived tissues and neoplasms, such as chordoma. However, the prognostic relevance of brachyury expression in chordoma is still unknown. The improvement of tissue microarray technology has provided the opportunity to perform analyses of tumor tissues on a large scale in a uniform and consistent manner. This study was designed with the use of tissue microarray to determine the expression of brachyury. Brachyury expression in chordoma tissues from 78 chordoma patients was analyzed by immunohistochemical staining of tissue microarray. The clinicopathologic parameters, including gender, age, location of tumor and metastatic status were evaluated. Fifty-nine of 78 (75.64%) tumors showed nuclear staining for brachyury, and among them, 29 tumors (49.15%) showed 1+ (mobile spine. However, there was no significant relationship between brachyury expression and other clinical variables. By Kaplan-Meier analysis, brachyury expression failed to produce any significant relationship with the overall survival rate. In conclusion, brachyury expression is not a prognostic indicator in chordoma.

  11. 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.

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

    International Nuclear Information System (INIS)

    Hofman, P.A.M.; Wilmink, J.T.

    1996-01-01

    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

  13. Intradural extramedullary spinal cord tumours: A retrospective study of sur­gical outcomes

    Directory of Open Access Journals (Sweden)

    Md. Kamrul Ahsan

    2016-07-01

    Full Text Available Background: Intradural extramedullary spinal cord tumours (IESCT accounts for approximately two thirds of all intraspinal neoplasm and are of important clinical consideration and surgery is the essence in cases with neurological deterioration.Objective: To share our experience on the outcome of surgical excision of intradural extramedullary spinal cord tumours. Methods: Results of 60 patients surgically treated intradural extramedullary spinal tumours between Octo­ber 2003 and October 2015 at Bangabandhu Sheikh Mujib Medical University and in our private settings, Dhaka, were analyzed retrospectively. There were 32 males, 28 females with an average age of 52.4 years (13-70 years and followed up for at least a year. The preoperative symptom with duration, tumours location and intradural space occupancy and the histopathological diagnosis were analyzed. Pain was evaluated by the visual analogue scale (VAS and the neurologic function was assessed by Nurick's grade.Results: The tumours were located as, thoracic 32 (53.33%, lumbar 16 (26.67%, cervical 04 (6.67%, and junctional 08 (13.33%, CervicoThoracic-01, Thoracolumbar-07. The histopathological diagnosis included schwannoma 35 (58.33%, meningiomas 14 (23.33%, neurofbroma 4 (6.67%, arachnoid cyst and myxopapillary ependymoma 03 (05.00% each and paraganglioma 01 (01.67%. The VAS score was reduced in all cases from 8.0 ± 1.2 to 1.2 ± 0.8 (p < 0.003 and the Nurick's grade was improved in all cases from 3.0 ± 1.3 to 1.0 ± 0.0 (p < 0.005. The preoperative neurological deficit improved within 8 postoperative weeks in most cases and within 1 postoperative year in all cases. Complications included cerebrospinal fluid leakage, parasthesia, dependant bedsore 02 (3.33% each and recurrence 03 (05.00%. and further neurological deterioration 1 (01.67% case.Conclusion: lntradural extramedullary tumors detected by MRI are mostly benign and good clinical results can be obtained when treated surgi

  14. 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.

  15. 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.

  16. 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

    2018-05-01

    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.

  17. 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.

  18. Transrectal EUS-guided FNA biopsy of a presacral chordoma-report of a case and review of the literature

    Institute of Scientific and Technical Information of China (English)

    Klaus Gottlieb; Paul H Lin; David M Liu; Karl Anders

    2008-01-01

    Chordomas are rare tumors which originate from the remnants of the notochord.These tumors are locally aggressive and have a predilection for the ends of the axial skeleton.An important prerequisite for optimal management of these tumors is a correct preoperative diagnosis.The present case is the first report of the use of endoscopic ultrasound to obtain transrectal fine needle aspiration biopsy of a presacral chordoma.A review of the prior computer tomography (CT) scans allowed us to calculate the tumor volume doubling time (18.3 mo).Transrectal biopsy of chordomas is controversial,however we believe that such concerns are not justified.

  19. 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

  20. Base of skull and cervical spine chordomas in children treated by high-dose irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Benk, Veronique; Liebsch, Norbert J; Munzenrider, John E; Efird, John; McManus, Patricia; Suit, Herman

    1995-02-01

    Purpose: To evaluate the outcome of children with base of skull or cervical spine chordomas treated by high dose irradiation. Methods and Materials: Eighteen children, 4 to 18 years of age, with base of skull or cervical spine chordomas, received fractionated high-dose postoperative radiation using mixed photon and 160 MeV proton beams. The median tumor dose was 69 Cobalt Gray-equivalent (CGE) with a 1.8 CGE daily fraction. Results: The median follow-up was 72 months. The 5-year actuarial survival was 68% and the 5-year disease-free survival (DFS) was 63%. The only significant prognostic factor was the location: patients with cervical spine chordomas had a worse survival than those with base of skull lesions (p = 0.008). The incidence of treatment-related morbidity was acceptable: two patients developed a growth hormone deficit corrected by hormone replacement, one temporal lobe necrosis, and one fibrosis of the temporalis muscle, improved by surgery. Conclusion: Chordomas in children behave similarly to those in adults: children can receive the same high-dose irradiation as adults with acceptable morbidity.

  1. Benign chordoma of the sacral bone. Radiologic appearance and differential dignosis

    International Nuclear Information System (INIS)

    Pegios, W.; Vogl, T.J.; Rausch, M.; Klein, U.; Balzer, J.O.; Hammerstingl, R.; Mack, M.G.; Felix, R.

    1994-01-01

    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.) [de

  2. Base of skull and cervical spine chordomas in children treated by high-dose irradiation

    International Nuclear Information System (INIS)

    Benk, Veronique; Liebsch, Norbert J.; Munzenrider, John E.; Efird, John; McManus, Patricia; Suit, Herman

    1995-01-01

    Purpose: To evaluate the outcome of children with base of skull or cervical spine chordomas treated by high dose irradiation. Methods and Materials: Eighteen children, 4 to 18 years of age, with base of skull or cervical spine chordomas, received fractionated high-dose postoperative radiation using mixed photon and 160 MeV proton beams. The median tumor dose was 69 Cobalt Gray-equivalent (CGE) with a 1.8 CGE daily fraction. Results: The median follow-up was 72 months. The 5-year actuarial survival was 68% and the 5-year disease-free survival (DFS) was 63%. The only significant prognostic factor was the location: patients with cervical spine chordomas had a worse survival than those with base of skull lesions (p = 0.008). The incidence of treatment-related morbidity was acceptable: two patients developed a growth hormone deficit corrected by hormone replacement, one temporal lobe necrosis, and one fibrosis of the temporalis muscle, improved by surgery. Conclusion: Chordomas in children behave similarly to those in adults: children can receive the same high-dose irradiation as adults with acceptable morbidity

  3. 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.

  4. 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

  5. Pediatric Clival Chordoma: A Curable Disease that Conforms to Collins' Law.

    Science.gov (United States)

    Rassi, Marcio S; Hulou, M Maher; Almefty, Kaith; Bi, Wenya Linda; Pravdenkova, Svetlana; Dunn, Ian F; Smith, Timothy R; Al-Mefty, Ossama

    2018-05-01

    Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined. To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins' law for embryonal tumors. Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263). Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival. Long-term overall and progression-free survival in children harboring skull base chordomas can be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins' law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure.

  6. 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

  7. Carbon ion radiotherapy for chordomas and low-grade chondrosarcomas of the skull base. Results in 67 patients

    Energy Technology Data Exchange (ETDEWEB)

    Schulz-Ertner, D.; Wannenmacher, M. [Dept. of Clinical Radiology, Univ. of Heidelberg (Germany); Nikoghosyan, A.; Thilmann, C.; Jaekel, O.; Karger, C. [German Cancer Research Center (dkfz), Heidelberg (Germany); Haberer, T.; Scholz, M.; Kraft, G. [Dept. of Biophysics, German Ion Research Center (GSI), Darmstadt (Germany); Debus, J. [Dept. of Clinical Radiology, Univ. of Heidelberg (Germany); German Cancer Research Center (dkfz), Heidelberg (Germany)

    2003-09-01

    Purpose: To prospectively evaluate outcome and toxicity after carbon ion radiotherapy (RT) in chordomas and low-grade chondrosarcomas. Patients and Methods: Between September 1998 and December 2001, 74 patients were treated for chordomas and chondrosarcomas with carbon ion RT at the ''Gesellschaft fuer Schwerionenforschung'' (GSI). Seven patients reirradiated with reduced carbon ion doses after conventional RT were excluded from the analysis, leaving 67 evaluable patients (44 chordomas and 23 chondrosarcomas) who received a full course of carbon ion therapy. Tumor-conform application of carbon ion beams was realized by intensity-controlled raster scanning with active energy variation. Three-dimensional treatment planning included intensity modulation and biological plan optimization. A median dose of 60 GyE was applied to the target volume within 20 consecutive days at a dose of 3.0 GyE per fraction. Results: Median follow-up was 15 months (range 3-46 months). At 3 years, actuarial local control was 100% for chondrosarcomas and 87% for chordomas, respectively. Partial tumor remission was observed in 14/44 (31%) chordoma patients and in 4/23 (17%) chondrosarcoma patients. At 3 years, actuarial overall survival was 100% for chondrosarcomas and 89% for chordomas, respectively. No severe side effects > CTC III have been observed. Conclusions: These data demonstrate the clinical efficiency and safety of scanning beam delivery of carbon ion beams in patients with skull base chordomas and chondrosarcomas. The observation of tumor regressions at a dose level of 60 GyE may indicate that the biological effectiveness of carbon ions in chordomas and chondrosarcomas is higher than initially estimated. (orig.)

  8. Carbon ion radiotherapy for chordomas and low-grade chondrosarcomas of the skull base. Results in 67 patients

    International Nuclear Information System (INIS)

    Schulz-Ertner, D.; Wannenmacher, M.; Nikoghosyan, A.; Thilmann, C.; Jaekel, O.; Karger, C.; Haberer, T.; Scholz, M.; Kraft, G.; Debus, J.

    2003-01-01

    Purpose: To prospectively evaluate outcome and toxicity after carbon ion radiotherapy (RT) in chordomas and low-grade chondrosarcomas. Patients and Methods: Between September 1998 and December 2001, 74 patients were treated for chordomas and chondrosarcomas with carbon ion RT at the ''Gesellschaft fuer Schwerionenforschung'' (GSI). Seven patients reirradiated with reduced carbon ion doses after conventional RT were excluded from the analysis, leaving 67 evaluable patients (44 chordomas and 23 chondrosarcomas) who received a full course of carbon ion therapy. Tumor-conform application of carbon ion beams was realized by intensity-controlled raster scanning with active energy variation. Three-dimensional treatment planning included intensity modulation and biological plan optimization. A median dose of 60 GyE was applied to the target volume within 20 consecutive days at a dose of 3.0 GyE per fraction. Results: Median follow-up was 15 months (range 3-46 months). At 3 years, actuarial local control was 100% for chondrosarcomas and 87% for chordomas, respectively. Partial tumor remission was observed in 14/44 (31%) chordoma patients and in 4/23 (17%) chondrosarcoma patients. At 3 years, actuarial overall survival was 100% for chondrosarcomas and 89% for chordomas, respectively. No severe side effects > CTC III have been observed. Conclusions: These data demonstrate the clinical efficiency and safety of scanning beam delivery of carbon ion beams in patients with skull base chordomas and chondrosarcomas. The observation of tumor regressions at a dose level of 60 GyE may indicate that the biological effectiveness of carbon ions in chordomas and chondrosarcomas is higher than initially estimated. (orig.)

  9. Sacrococcygeal chordoma: increased 99mTc methylene diphosphonate uptake on single photon emission computed tomography/computed tomography bone scintigraphy

    International Nuclear Information System (INIS)

    Kamaleshwaran, Koramadai Karuppuswamy; Bhattacharya, Anish; Harisankar, Chidambaram Natarajan Balasubramaniam; Mittal, Bhagwant Rai; Goni, Vijay

    2012-01-01

    Chordoma is a malignant tumor arising from the remnants of the notochord, and is the most frequent primitive tumor of the sacrum. While most sacral tumors show increased concentration of bone-seeking radiopharmaceuticals, chordomas usually exhibit decreased uptake. The authors present an image of a sacrococcygeal chordoma with osteolysis and increased uptake of 99m Tc methylene diphosphonate on planar and single photon emission computed tomography/computed tomography bone scintigraphy. (author)

  10. FAS/FASL are dysregulated in chordoma and their loss-of-function impairs zebrafish notochord formation.

    Science.gov (United States)

    Ferrari, Luca; Pistocchi, Anna; Libera, Laura; Boari, Nicola; Mortini, Pietro; Bellipanni, Gianfranco; Giordano, Antonio; Cotelli, Franco; Riva, Paola

    2014-07-30

    Chordoma is a rare malignant tumor that recapitulates the notochord phenotype and is thought to derive from notochord remnants not correctly regressed during development. Apoptosis is necessary for the proper notochord development in vertebrates, and the apoptotic pathway mediated by Fas and Fasl has been demonstrated to be involved in notochord cells regression. This study was conducted to investigate the expression of FAS/FASL pathway in a cohort of skull base chordomas and to analyze the role of fas/fasl homologs in zebrafish notochord formation. FAS/FASL expression was found to be dysregulated in chordoma leading to inactivation of the downstream Caspases in the samples analyzed. Both fas and fasl were specifically expressed in zebrafish notochord sorted cells. fas and fasl loss-of-function mainly resulted in larvae with notochord multi-cell-layer jumps organization, larger vacuolated notochord cells, defects in the peri-notochordal sheath structure and in vertebral mineralization. Interestingly, we observed the persistent expression of ntla and col2a1a, the zebrafish homologs of the human T gene and COL2A1 respectively, which are specifically up-regulated in chordoma. These results demonstrate for the first time the dysregulation of FAS/FASL in chordoma and their role in notochord formation in the zebrafish model, suggesting their possible implication in chordoma onset.

  11. A New Classification for Pathologies of Spinal Meninges-Part 2: Primary and Secondary Intradural Arachnoid Cysts.

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    Klekamp, Jörg

    2017-08-01

    Spinal intradural arachnoid cysts are rare causes of radiculopathy or myelopathy. Treatment options include resection, fenestration, or cyst drainage. To classify intradural spinal arachnoid cysts and present results of their treatment. Among 1519 patients with spinal space occupying lesions, 130 patients demonstrated intradural arachnoid cysts. Neuroradiological and surgical features were reviewed and clinical data analyzed. Twenty-one patients presented arachnoid cysts as a result of an inflammatory leptomeningeal reaction related to meningitis, subarachnoid hemorrhage, intrathecal injections, intradural surgery, or trauma, ie, secondary cysts. For the remaining 109 patients, no such history could be elucidated, ie, primary cysts. Forty-six percent of primary and 86% of secondary cysts were associated with syringomyelia. Patients presented after an average history of 53 ± 88 months. There were 122 thoracic and 7 lumbar cysts plus 1 cervical cyst. Fifty-nine patients with primary and 15 patients with secondary cysts underwent laminotomies with complete or partial cyst resection and duraplasty. Mean follow-up was 57 ± 52 months. In the first postoperative year, profound improvements for primary cysts were noted, in contrast to marginal changes for secondary cysts. Progression-free survival for 10 years following surgery was determined as 83% for primary compared to 15% for secondary cysts. Despite differences in clinical presentation, progression-free survival was almost identical for patients with or without syringomyelia. Complete or partial resection leads to favorable short- and long-term results for primary arachnoid cysts. For secondary cysts, surgery can only provide clinical stabilization for a limited time due to the often extensive arachnoiditis. Copyright © 2017 by the Congress of Neurological Surgeons

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

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    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.

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

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    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

  14. Enhanced killing of chordoma cells by antibody-dependent cell-mediated cytotoxicity employing the novel anti-PD-L1 antibody avelumab.

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    Fujii, Rika; Friedman, Eitan R; Richards, Jacob; Tsang, Kwong Y; Heery, Christopher R; Schlom, Jeffrey; Hodge, James W

    2016-06-07

    Chordoma, a rare bone tumor derived from the notochord, has been shown to be resistant to conventional therapies. Checkpoint inhibition has shown great promise in immune-mediated therapy of diverse cancers. The anti-PD-L1 mAb avelumab is unique among checkpoint inhibitors in that it is a fully human IgG1 capable of mediating antibody-dependent cell-mediated cytotoxicity (ADCC) of PD-L1-expressing tumor cells. Here, we investigated avelumab as a potential therapy for chordoma. We examined 4 chordoma cell lines, first for expression of PD-L1, and in vitro for ADCC killing using NK cells and avelumab. PD-L1 expression was markedly upregulated by IFN-γ in all 4 chordoma cell lines, which significantly increased sensitivity to ADCC. Brachyury is a transcription factor that is uniformly expressed in chordoma. Clinical trials are ongoing in which chordoma patients are treated with brachyury-specific vaccines. Co-incubating chordoma cells with brachyury-specific CD8+ T cells resulted in significant upregulation of PD-L1 on the tumor cells, mediated by the CD8+ T cells' IFN-γ production, and increased sensitivity of chordoma cells to avelumab-mediated ADCC. Residential cancer stem cell subpopulations of chordoma cells were also killed by avelumab-mediated ADCC to the same degree as non-cancer stem cell populations. These findings suggest that as a monotherapy for chordoma, avelumab may enable endogenous NK cells, while in combination with T-cell immunotherapy, such as a vaccine, avelumab may enhance NK-cell killing of chordoma cells via ADCC.

  15. Zoledronic acid in metastatic chondrosarcoma and advanced sacrum chordoma: two case reports

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    Capasso Elena

    2009-01-01

    Full Text Available Abstract Introduction Chondrosarcomas and chordomas are usually chemoresistant bone tumors and may have a poor prognosis when advanced. They are usually associated with worsening pain difficult to control. Patients and Methods Zoledronic acid was used in a 63-year-old man with metastatic chondrosarcoma and in a 66-year-old woman with a diagnosis of sacrum chordoma both reporting severe pain related to tumor. Results In the first case, zoledronic acid was able to maintain pain control despite disease progression following chemotherapy, in the other case, zoledronic acid only produced significant clinical benefit. Conclusion Control of pain associated with bone tumors such as chondrosarcoma and chondroma may significantly improve from use of zoledronic acid, independently from tumor response to other treatments. Evaluation on larger series are needed to confirm the clinical effect of this bisphosphonate on such tumors.

  16. Malignant transformation of clival chordoma after gamma knife surgery. Case report

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    Tsuboi, Yoshifumi; Hayashi, Nakamasa; Kurimoto, Masanori; Nagai, Shoichi; Sasahara, Masakiyo; Endo, Shunro

    2007-01-01

    A 54-year-old woman presented a midline clival tumor manifesting as right abducens palsy in May 1997. Magnetic resonance (MR) imaging revealed a midline clival tumor. She underwent surgery twice with the transsphenoidal approach and gamma knife surgery for residual tumor. The histological diagnosis was chordoma. MR imaging revealed that the tumor had extended to the right cerebellopontine angle, with spinal seeding in February 2002. She underwent partial removal of the right cerebellopontine angle tumor. The histological diagnosis was chordoma with slight nuclear atypism. She died 5 years and 5 months after the first gamma knife surgery. Autopsy revealed multiple areas of spinal seeding. Histological examination confirmed malignant transformation with unique epithelial characteristics, possibly caused by gamma knife surgery. (author)

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

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    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.

  18. Image-guided, intensity-modulated radiation therapy (IG-IMRT) for skull base chordoma and chondrosarcoma: preliminary outcomes.

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    Sahgal, Arjun; Chan, Michael W; Atenafu, Eshetu G; Masson-Cote, Laurence; Bahl, Gaurav; Yu, Eugene; Millar, Barbara-Ann; Chung, Caroline; Catton, Charles; O'Sullivan, Brian; Irish, Jonathan C; Gilbert, Ralph; Zadeh, Gelareh; Cusimano, Michael; Gentili, Fred; Laperriere, Normand J

    2015-06-01

    We report our preliminary outcomes following high-dose image-guided intensity modulated radiotherapy (IG-IMRT) for skull base chordoma and chondrosarcoma. Forty-two consecutive IG-IMRT patients, with either skull base chordoma (n = 24) or chondrosarcoma (n = 18) treated between August 2001 and December 2012 were reviewed. The median follow-up was 36 months (range, 3-90 mo) in the chordoma cohort, and 67 months (range, 15-125) in the chondrosarcoma cohort. Initial surgery included biopsy (7% of patients), subtotal resection (57% of patients), and gross total resection (36% of patients). The median IG-IMRT total doses in the chondrosarcoma and chordoma cohorts were 70 Gy and 76 Gy, respectively, delivered with 2 Gy/fraction. For the chordoma and chondrosarcoma cohorts, the 5-year overall survival and local control rates were 85.6% and 65.3%, and 87.8% and 88.1%, respectively. In total, 10 patients progressed locally: 8 were chordoma patients and 2 chondrosarcoma patients. Both chondrosarcoma failures were in higher-grade tumors (grades 2 and 3). None of the 8 patients with grade 1 chondrosarcoma failed, with a median follow-up of 77 months (range, 34-125). There were 8 radiation-induced late effects-the most significant was a radiation-induced secondary malignancy occurring 6.7 years following IG-IMRT. Gross total resection and age were predictors of local control in the chordoma and chondrosarcoma patients, respectively. We report favorable survival, local control and adverse event rates following high dose IG-IMRT. Further follow-up is needed to confirm long-term efficacy. © The Author(s) 2014. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  19. Resolving tumor heterogeneity: genes involved in chordoma cell development identified by low-template analysis of morphologically distinct cells.

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    Amin El-Heliebi

    Full Text Available The classical sacrococcygeal chordoma tumor presents with a typical morphology of lobulated myxoid tumor tissue with cords, strands and nests of tumor cells. The population of cells consists of small non-vacuolated cells, intermediate cells with a wide range of vacuolization and large heavily vacuolated (physaliferous cells. To date analysis was only performed on bulk tumor mass because of its rare incidence, lack of suited model systems and technical limitations thereby neglecting its heterogeneous composition. We intended to clarify whether the observed cell types are derived from genetically distinct clones or represent different phenotypes. Furthermore, we aimed at elucidating the differences between small non-vacuolated and large physaliferous cells on the genomic and transcriptomic level. Phenotype-specific analyses of small non-vacuolated and large physaliferous cells in two independent chordoma cell lines yielded four candidate genes involved in chordoma cell development. UCHL3, coding for an ubiquitin hydrolase, was found to be over-expressed in the large physaliferous cell phenotype of MUG-Chor1 (18.7-fold and U-CH1 (3.7-fold cells. The mannosyltransferase ALG11 (695-fold and the phosphatase subunit PPP2CB (18.6-fold were found to be up-regulated in large physaliferous MUG-Chor1 cells showing a similar trend in U-CH1 cells. TMEM144, an orphan 10-transmembrane family receptor, yielded contradictory data as cDNA microarray analysis showed up- but RT-qPCR data down-regulation in large physaliferous MUG-Chor1 cells. Isolation of few but morphologically identical cells allowed us to overcome the limitations of bulk analysis in chordoma research. We identified the different chordoma cell phenotypes to be part of a developmental process and discovered new genes linked to chordoma cell development representing potential targets for further research in chordoma tumor biology.

  20. The role of radiosurgery in the management of chordoma and chondrosarcoma of the cranial base

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    Kondziolka, D.; Lunsford, L.D.; Flickinger, J.C.

    1991-01-01

    Despite conventional multimodality treatment (surgery and fractionated radiation therapy), recurrence and clinical progression of cranial base chordomas and chondrosarcomas are common. The malignant behavior of these tumors is a result of their critical location, locally aggressive nature, and high recurrence rate. To explore the role of radiosurgery in the treatment of these skull base neoplasms, we assessed its use in four patients with chordoma and two with chondrosarcoma. In five of the patients, radiosurgery was used as adjuvant therapy for residual or recurrent tumors after surgical debulking, and in one patient with a chordoma, it was the primary treatment. No patient received fractionated external beam radiotherapy. All tumors were less than 30 mm in diameter and were treated with 20 Gy to the tumor margin. Skull base computed tomography and magnetic resonance images were essential to define the anatomic relationships between tumor and adjacent basal structures. During follow-up (mean, 22 mo; range, 8-36 mo), the authors found no progression of the treated tumor volume in any patient. Neurological deficits before treatment improved in three patients; the other three patients remained in stable neurological condition. Serial follow-up imaging studies demonstrated that two patients showed reduction in tumor size and four patients had no tumor growth. In one patient, a metastatic parietal lobe chondrosarcoma developed and was treated by microsurgery. Another patient showed tumor progression outside of the radiosurgical treatment volume. The authors results attest to the value of stereotactic radiosurgery as an adjuvant or primary treatment for selected patients with chordoma or chondrosarcoma and demonstrate its potential advantages over standard fractionated irradiation. Analysis of the long-term clinical and imaging effects after radiosurgery is warranted

  1. Value of MRI in the diagnosis of non-clival, non-sacral chordoma

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    Smolders, D.; Wang, X.; Vanhoenacker, F.; De Schepper, A.M. [Department of Radiology, University Hospital Antwerp, Wilrijkstraat 10, 2650, Edegem (Belgium); Drevelengas, A. [Department of Radiology, University of Thessaloniki, Thessaloniki (Greece)

    2003-06-01

    To determine the MR features of non-sacral, non-clival chordoma and to describe a MR prototype of the lesion.Design and patients We reviewed the MR findings of 10 patients with a histologically proven chordoma (6 cervical spine, 1 thoracic spine, 3 lumbar spine). There were three female and seven male patients. Age ranged from 12 to 66 years with a mean age of 44.6 years. The MR images were reviewed for signal intensity (SI) and morphology. All lesions showed a soft tissue extension spanning several vertebral segments. Most of the lesions exhibited a so-called collar button appearance (sagittal images). Two cases of cervical chordoma displayed a ''dumbbell morphology'' (axial images) or ''mushroom'' appearance without bone involvement and with enlargement of the neuroforamen mimicking a neurogenic tumor. Although the region of the nucleus pulposus is the last part of the fetal notochord in the adult to involute, disks were surprisingly spared in all patients. Eight of 10 patients showed heterogeneous SI on all sequences. The overall SI of all lesions was isointense or slightly higher than that of muscle on T1-weighted images. All lesions exhibited high SI on T2-weighted images. After gadolinium contrast administration there was a moderate enhancement in most cases. Although the SI on MR imaging is not specific, chordoma should be considered when a destructive lesion of a vertebral body is associated with a soft tissue mass with a collar button or mushroom appearance and dumbbell morphology, spanning several vertebral segments and sparing the disk(s). (orig.)

  2. Value of MRI in the diagnosis of non-clival, non-sacral chordoma

    International Nuclear Information System (INIS)

    Smolders, D.; Wang, X.; Vanhoenacker, F.; De Schepper, A.M.; Drevelengas, A.

    2003-01-01

    To determine the MR features of non-sacral, non-clival chordoma and to describe a MR prototype of the lesion.Design and patients We reviewed the MR findings of 10 patients with a histologically proven chordoma (6 cervical spine, 1 thoracic spine, 3 lumbar spine). There were three female and seven male patients. Age ranged from 12 to 66 years with a mean age of 44.6 years. The MR images were reviewed for signal intensity (SI) and morphology. All lesions showed a soft tissue extension spanning several vertebral segments. Most of the lesions exhibited a so-called collar button appearance (sagittal images). Two cases of cervical chordoma displayed a ''dumbbell morphology'' (axial images) or ''mushroom'' appearance without bone involvement and with enlargement of the neuroforamen mimicking a neurogenic tumor. Although the region of the nucleus pulposus is the last part of the fetal notochord in the adult to involute, disks were surprisingly spared in all patients. Eight of 10 patients showed heterogeneous SI on all sequences. The overall SI of all lesions was isointense or slightly higher than that of muscle on T1-weighted images. All lesions exhibited high SI on T2-weighted images. After gadolinium contrast administration there was a moderate enhancement in most cases. Although the SI on MR imaging is not specific, chordoma should be considered when a destructive lesion of a vertebral body is associated with a soft tissue mass with a collar button or mushroom appearance and dumbbell morphology, spanning several vertebral segments and sparing the disk(s). (orig.)

  3. Clival Ectopic Pituitary Adenoma Mimicking a Chordoma: Case Report and Review of the Literature

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    Constantine L. Karras

    2016-01-01

    Full Text Available Background. Purely ectopic pituitary adenomas are exceedingly rare. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Case Presentation. A 41-year-old male presented with an incidental clival lesion presumed to be a chordoma. On MRI it involved the entire clivus, extended laterally to the petroclival junction, and invaded the cavernous sinuses bilaterally, encasing both internal carotid arteries, without direct extension into the sella. Intraoperatively, it was clear that the tumor originated from the clivus and that the sellar dura was completely intact. Frozen-section pathology was consistent with a pituitary adenoma. Immunostaining was positive for synaptophysin and prolactin with a low Ki-67 index, suggestive of a prolactinoma. Additional immunohistochemical stains seen in chordomas (EMA, S100, and Brachyury and other metastatic tumors were negative. A postoperative endocrine workup revealed an elevated serum prolactin of 881.3 ng/mL (normal < 20. Conclusions. In conclusion, it is crucial to maintain an extensive differential diagnosis when evaluating a patient with a clival lesion. Ectopic clival pituitary adenomas, although rare, may warrant an endocrinological workup preoperatively as the majority may respond to medical treatment.

  4. On the cost-effectiveness of Carbon ion radiation therapy for skull base chordoma

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    Jaekel, Oliver; Land, Beate; Combs, Stephanie Elisabeth; Schulz-Ertner, Daniela; Debus, Juergen

    2007-01-01

    Aim: The cost-effectiveness of Carbon ion radiotherapy (RT) for patients with skull base chordoma is analyzed. Materials and Methods: Primary treatment costs and costs for recurrent tumors are estimated. The costs for treatment of recurrent tumors were estimated using a sample of 10 patients presenting with recurrent chordoma at the base of skull at DKFZ. Using various scenarios for the local control rate and reimbursements of Carbon ion therapy the cost-effectiveness of ion therapy for these tumors is analyzed. Results: If local control rate for skull base chordoma achieved with carbon ion therapy exceeds 70.3%, the overall treatment costs for carbon RT are lower than for conventional RTI. The cost-effectiveness ratio for carbon RT is 2539 Euro per 1% increase in survival, or 7692 Euro per additional life year. Conclusion: Current results support the thesis that Carbon ion RT, although more expensive, is at least as cost-effective as advanced photon therapies for these patients. Ion RT, however, offers substantial benefits for the patients such as improved control rates and less severe side effects

  5. Intradural Solitary Fibrous Tumor of the Lumbar Spine: A Distinctive Case Report

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    Recep Basaran

    2015-01-01

    Full Text Available Background. Solitary fibrous tumors are ubiquitous mesenchymal neoplasms of putative fibroblastic origin. They were originally described in the pleura but subsequently have been reported in many extraserosal sites. Solitary fibrous tumors may also occur in the meninges, central nervous system parenchyma, and spinal cord. Case. A 67-year-old male patient with progressive lower extremity weakness, urinary urgency, and sexual dysfunction has been admitted to our hospital. On his lumbar MRI, we detected an intradural lesion posterior to the L3 vertebral corpus. We resected the lesion by L3 total laminectomy. Immunohistological findings revealed strong and diffuse immunopositivity with vimentin, CD34, and bcl-2. Ki-67 proliferation index was 5–8%. We did not detect any recurrence 12 months after his operation. Conclusion. SFT is mostly seen in young and middle-aged patients and should be considered among differential diagnosis in cases suffering from pain, hypoesthesia, and urinary dysfunction. Gross total resection should be primary treatment. Tumors that have high Ki-67 labeling should be followed up for potential recurrences.

  6. Microsurgical and Endoscopic Anatomy for Intradural Temporal Bone Drilling and Applications of the Electromagnetic Navigation System: Various Extensions of the Retrosigmoid Approach.

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    Matsushima, Ken; Komune, Noritaka; Matsuo, Satoshi; Kohno, Michihiro

    2017-07-01

    The use of the retrosigmoid approach has recently been expanded by several modifications, including the suprameatal, transmeatal, suprajugular, and inframeatal extensions. Intradural temporal bone drilling without damaging vital structures inside or beside the bone, such as the internal carotid artery and jugular bulb, is a key step for these extensions. This study aimed to examine the microsurgical and endoscopic anatomy of the extensions of the retrosigmoid approach and to evaluate the clinical feasibility of an electromagnetic navigation system during intradural temporal bone drilling. Five temporal bones and 8 cadaveric cerebellopontine angles were examined to clarify the anatomy of retrosigmoid intradural temporal bone drilling. Twenty additional cerebellopontine angles were dissected in a clinical setting with an electromagnetic navigation system while measuring the target registration errors at 8 surgical landmarks on and inside the temporal bone. Retrosigmoid intradural temporal bone drilling expanded the surgical exposure to allow access to the petroclival and parasellar regions (suprameatal), internal acoustic meatus (transmeatal), upper jugular foramen (suprajugular), and petrous apex (inframeatal). The electromagnetic navigation continuously guided the drilling without line of sight limitation, and its small devices were easily manipulated in the deep and narrow surgical field in the posterior fossa. Mean target registration error was less than 0.50 mm during these procedures. The combination of endoscopic and microsurgical techniques aids in achieving optimal exposure for retrosigmoid intradural temporal bone drilling. The electromagnetic navigation system had clear advantages with acceptable accuracy including the usability of small devices without line of sight limitation. Copyright © 2017 Elsevier Inc. All rights reserved.

  7. Incidence, treatment, and survival patterns for sacral chordoma in the United States, 1974-2011

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    Esther Yu

    2016-09-01

    Full Text Available IntroductionSacral chordomas represent one half of all chordomas, a rare neoplasm of notochordal remnants. Current NCCN guidelines recommend surgical resection with or without adjuvant radiotherapy, or definitive radiation for unresectable cases. Recent advances in radiation for chordomas include conformal photon and proton beam radiation. We investigated incidence, treatment, and survival outcomes to observe any trends in response to improvements in surgical and radiation techniques over a near 40 year time period.Materials and Methods345 microscopically confirmed cases of sacral chordoma were identified between 1974 and 2011 from the Surveillance, Epidemiology, and End Results (SEER program of the National Cancer Institute. Cases were divided into three cohorts by calendar year, 1974-1989, 1990-1999, and 2000-2011, as well as into two groups by age less than or equal to 65 versus greater than 65 to investigate trends over time and age via Chi-square analysis. Kaplan-Meier analyses were performed to determine effects of treatment on survival. Multivariate Cox regression analysis was performed to determine predictors of overall survival.Results5-year overall survival for the entire cohort was 60.0%. Overall survival correlated significantly with treatment modality, with 44% surviving at 5 years with no treatment, 52% with radiation alone, 82% surgery alone, and 78% surgery and radiation (p<.001. Age greater than 65 was significantly associated with non-surgical management with radiation alone or no treatment (p<.001. Relatively fewer patients received radiation between 2000 and 2011 compared to prior time periods (p=.03 versus surgery, for which rates which did not vary significantly over time (p=.55. However, 5-year overall survival was not significantly different by time period. Age group and treatment modality were predictive for overall survival on multivariate analysis (p<.001. ConclusionSurgery remains an important component in the

  8. MicroRNA-608 and microRNA-34a regulate chordoma malignancy by targeting EGFR, Bcl-xL and MET.

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    Ying Zhang

    Full Text Available Chordomas are rare malignant tumors that originate from the notochord remnants and occur in the skull base, spine and sacrum. Due to a very limited understanding of the molecular pathogenesis of chordoma, there are no adjuvant and molecular therapies besides surgical resection and radiation therapy. microRNAs (miRNAs are small noncoding regulatory RNA molecules with critical roles in cancer. The role of miRNAs in chordomas is mostly unknown. We uncover microRNA-608 (miR-608 and microRNA-34a (miR-34a as novel tumor suppressive microRNAs that regulate malignancy in chordoma. We find that miR-608 and miR-34a expressions are downregulated in human chordoma cell lines and primary cells at least partially via alteration of their genes' copy numbers. We identify the commonly deregulated oncogenes EGFR and Bcl-xL as direct targets of miR-608 and the receptor tyrosine kinase MET as direct target of miR-34a. We show that EGFR and MET activations promote chordoma cell proliferation and invasion and that pharmacological inhibition of EGFR and MET inhibits chordoma cell proliferation and survival. We demonstrate that restoration of miR-608 and miR-34a inhibits cell proliferation and invasion and induces apoptosis in chordoma cells. We find that miR-34a inversely correlates with MET expression and miR-608 inversely correlates with EGFR expression in chordoma cells. These findings demonstrate for the first time that miR-608 and miR-34a regulate chordoma malignancy by regulating EGFR, MET and Bcl-xL.

  9. Analysis of prognostic factors for survival in patients with primary spinal chordoma using the SEER Registry from 1973 to 2014.

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    Pan, Yue; Lu, Lingyun; Chen, Junquan; Zhong, Yong; Dai, Zhehao

    2018-04-06

    Spinal chordomas are rare primary osseous tumors that arise from the remnants of the notochord. They are commonly considered slow-growing, locally invasive neoplasms with little tendency to metastasize, but the high recurrent rate of spinal chordomas may seriously affect the survival rate and quality of life of patients. The aim of the study is to describe the epidemiological data and determine the prognostic factors for decreased survival in patients with primary spinal chordoma. The Surveillance, Epidemiology, and End Results (SEER) Registry database, a US population-based cancer registry database, was used to identify all patients diagnosed with primary spinal chordoma from 1973 to 2014. We utilized Kaplan-Meier method and Cox proportional hazards regression analysis to evaluate the association between patients overall survival and relevant characteristics, including age, gender, race, disease stage, treatment methods, primary tumor site, marital status, and urban county background. In the data set between 1973 and 2014, a total of 808 patients were identified with primary spinal chordoma. The overall rate of distant metastatic cases in our cohort was only 7.7%. Spinal chordoma was more common occurred in men (62.6%) than women (37.3%). Majority of neoplasms were found in the White (87.9%), while the incidence of the Black is relatively infrequent (3.3%). Three hundred fifty-seven spinal chordomas (44.2%) were located in the vertebral column, while 451 patients' tumor (55.8%) was located in the sacrum or pelvis. Age ≥ 60 years (HR = 2.72; 95%CI, 1.71 to 2.89), distant metastasis (HR = 2.16; 95%CI, 1.54 to 3.02), and non-surgical therapy (HR = 2.14; 95%CI, 1.72 to 2.69) were independent risk factors for survival reduction in analysis. Survival did not significantly differ as a factor of tumor site (vertebrae vs sacrum/pelvis) for primary spinal chordoma (HR = 0.93, P = 0.16). Race (P = 0.52), gender (P = 0.11), marital status (P = 0.94), and

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

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    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.

  11. Proton beam therapy in the management of skull base chordomas: systematic review of indications, outcomes, and implications for neurosurgeons.

    Science.gov (United States)

    Matloob, Samir A; Nasir, Haleema A; Choi, David

    2016-08-01

    Chordomas are rare tumours affecting the skull base. There is currently no clear consensus on the post-surgical radiation treatments that should be used after maximal tumour resection. However, high-dose proton beam therapy is an accepted option for post-operative radiotherapy to maximise local control, and in the UK, National Health Service approval for funding abroad is granted for specific patient criteria. To review the indications and efficacy of proton beam therapy in the management of skull base chordomas. The primary outcome measure for review was the efficacy of proton beam therapy in the prevention of local occurrence. A systematic review of English and non-English articles using MEDLINE (1946-present) and EMBASE (1974-present) databases was performed. Additional studies were reviewed when referenced in other studies and not available on these databases. Search terms included chordoma or chordomas. The PRISMA guidelines were followed for reporting our findings as a systematic review. A total of 76 articles met the inclusion and exclusion criteria for this review. Limitations included the lack of documentation of the extent of primary surgery, tumour size, and lack of standardised outcome measures. Level IIb/III evidence suggests proton beam therapy given post operatively for skull base chordomas results in better survival with less damage to surrounding tissue. Proton beam therapy is a grade B/C recommended treatment modality for post-operative radiation therapy to skull base chordomas. In comparison to other treatment modalities long-term local control and survival is probably improved with proton beam therapy. Further, studies are required to directly compare proton beam therapy to other treatment modalities in selected patients.

  12. Intradural approach to selective stimulation in the spinal cord for treatment of intractable pain: design principles and wireless protocol

    Science.gov (United States)

    Howard, M. A.; Utz, M.; Brennan, T. J.; Dalm, B. D.; Viljoen, S.; Jeffery, N. D.; Gillies, G. T.

    2011-08-01

    We introduce an intradural approach to spinal cord stimulation for the relief of intractable pain, and describe the biophysical rationale that underlies its design and performance requirements. The proposed device relies on wireless, inductive coupling between a pial surface implant and its epidural controller, and we present the results of benchtop experiments that demonstrate the ability to transmit and receive a frequency-modulated 1.6 MHz carrier signal between micro-coil antennae scaled to the ≈ 1 cm dimensions of the implant, at power levels of about 5 mW. Plans for materials selection, microfabrication, and other aspects of future development are presented and discussed.

  13. Diagnosing an extra-axial chordoma of the proximal tibia with the help of brachyury, a molecule required for notochordal differentiation

    Energy Technology Data Exchange (ETDEWEB)

    O' Donnell, Paul [Royal National Orthopaedic Hospital, Department of Radiology, Stanmore, Middlesex (United Kingdom); University College London, Institute of Orthopaedics and Musculoskeletal Science, Stanmore, Middlesex (United Kingdom); Tirabosco, Roberto [Royal National Orthopaedic Hospital, Department of Histopathology, Stanmore, Middlesex (United Kingdom); Vujovic, Sonja; Henderson, Stephen; Boshoff, Chris [University College London, Wolfson Institute for Biomedical Research, London (United Kingdom); Bartlett, William; Briggs, Timothy W.R. [Royal National Orthopaedic Hospital, Department of Orthopaedic Surgery, Stanmore, Middlesex (United Kingdom); Flanagan, Adrienne M. [Royal National Orthopaedic Hospital, Department of Histopathology, Stanmore, Middlesex (United Kingdom); University College London, Institute of Orthopaedics and Musculoskeletal Science, Stanmore, Middlesex (United Kingdom)

    2007-01-15

    Chordomas are rare malignant bone tumours considered to arise from notochordal remnants that persist in the axial skeleton. Although their morphology can resemble that of a carcinoma, chondrosarcoma or malignant melanoma, the axial location and their well-defined immunophenotype, including expression of cytokeratins (CK7/20/8/18/19) and S100, generally allow the diagnosis to be made with confidence once the possibility is considered. In contrast, making a robust diagnosis of an extra-axial chordoma has been difficult in the absence of specific markers for chordomas. We have recently shown in gene expression microarray and immunohistochemistry studies that brachyury, a transcription factor crucial for notochordal development, is a specific and sensitive maker for chordomas. We now present a case of an intracortical tibial tumour, with detailed report of the imaging, and morphological features consistent with a chordoma, where notochordal differentiation was demonstrated with an antibody to brachyury. The tumour cells were also positive for cytokeratins, including CK19, and S100, CEA, EMA and HMBE1, findings which support the diagnosis of chordoma. Brachyury can be employed as a marker of notochordal differentiation and help identify confidently, for the first time, extra-axial bone and soft tissue chordomas, and tumours which may show focal notochordal differentiation. (orig.)

  14. Diagnosing an extra-axial chordoma of the proximal tibia with the help of brachyury, a molecule required for notochordal differentiation

    International Nuclear Information System (INIS)

    O'Donnell, Paul; Tirabosco, Roberto; Vujovic, Sonja; Henderson, Stephen; Boshoff, Chris; Bartlett, William; Briggs, Timothy W.R.; Flanagan, Adrienne M.

    2007-01-01

    Chordomas are rare malignant bone tumours considered to arise from notochordal remnants that persist in the axial skeleton. Although their morphology can resemble that of a carcinoma, chondrosarcoma or malignant melanoma, the axial location and their well-defined immunophenotype, including expression of cytokeratins (CK7/20/8/18/19) and S100, generally allow the diagnosis to be made with confidence once the possibility is considered. In contrast, making a robust diagnosis of an extra-axial chordoma has been difficult in the absence of specific markers for chordomas. We have recently shown in gene expression microarray and immunohistochemistry studies that brachyury, a transcription factor crucial for notochordal development, is a specific and sensitive maker for chordomas. We now present a case of an intracortical tibial tumour, with detailed report of the imaging, and morphological features consistent with a chordoma, where notochordal differentiation was demonstrated with an antibody to brachyury. The tumour cells were also positive for cytokeratins, including CK19, and S100, CEA, EMA and HMBE1, findings which support the diagnosis of chordoma. Brachyury can be employed as a marker of notochordal differentiation and help identify confidently, for the first time, extra-axial bone and soft tissue chordomas, and tumours which may show focal notochordal differentiation. (orig.)

  15. 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

  16. Challenges in Linear Accelerator Radiotherapy for Chordomas and Chondrosarcomas of the Skull Base: Focus on Complications

    Energy Technology Data Exchange (ETDEWEB)

    Hauptman, Jason S., E-mail: jhauptman@mednet.ucla.edu [Division of Stereotactic and Functional Neurosurgery, Department of Neurosurgery, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA (United States); Barkhoudarian, Garni; Safaee, Michael; Gorgulho, Alessandra [Division of Stereotactic and Functional Neurosurgery, Department of Neurosurgery, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA (United States); Tenn, Steven; Agazaryan, Nzhde; Selch, Michael [Department of Radiation Oncology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA (United States); De Salles, Antonio A.F. [Division of Stereotactic and Functional Neurosurgery, Department of Neurosurgery, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA (United States); Department of Radiation Oncology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA (United States)

    2012-06-01

    Purpose: Intracranial chordomas and chondrosarcomas are histologically low-grade, locally invasive tumors that infiltrate the skull base. Currently, consensus therapy includes surgical resection and adjuvant radiotherapy. Radiation delivery is typically limited by the proximity of these tumors to critical skull base structures. Methods: This is a retrospective review of 13 cases of chordomas and 2 cases of chondroid chondrosarcomas of the skull based treated with linear accelerator stereotactic radiotherapy (SRT, n = 10) or stereotactic radiosurgery (SRS, n = 5). The average time to the most recent follow-up visit was 4.5 years. The tumor characteristics, treatment details, and outcomes were recorded. Each radiation plan was reviewed, and the dosage received by the brainstem, optic apparatus, and pituitary was calculated. Results: Of the 10 patients treated with SRT, 6 were found to have unchanged or decreased tumor size as determined from radiographic follow-up. Of the 5 patients treated with SRS, 3 were found to have stable or unchanged tumors at follow-up. The complications included 1 SRT patient who developed endocrinopathy, 2 patients (1 treated with SRS and the other with SRT), who developed cranial neuropathy, and 1 SRS patient who developed visual deficits. Additionally, 1 patient who received both SRS and SRT within 2 years for recurrence experienced transient medial temporal lobe radiation changes that resolved. Conclusions: Where proton beam therapy is unavailable, linear accelerator-based SRT or radiosurgery remains a safe option for adjuvant therapy of chordomas and chondrosarcomas of the skull base. The exposure of the optic apparatus, pituitary stalk, and brainstem must be considered during planning to minimize complications. If the optic apparatus is included in the 80% isodose line, it might be best to fractionate therapy. Exposure of the pituitary stalk should be kept to <30 Gy to minimize endocrine dysfunction. Brainstem exposure should be

  17. Challenges in Linear Accelerator Radiotherapy for Chordomas and Chondrosarcomas of the Skull Base: Focus on Complications

    International Nuclear Information System (INIS)

    Hauptman, Jason S.; Barkhoudarian, Garni; Safaee, Michael; Gorgulho, Alessandra; Tenn, Steven; Agazaryan, Nzhde; Selch, Michael; De Salles, Antonio A.F.

    2012-01-01

    Purpose: Intracranial chordomas and chondrosarcomas are histologically low-grade, locally invasive tumors that infiltrate the skull base. Currently, consensus therapy includes surgical resection and adjuvant radiotherapy. Radiation delivery is typically limited by the proximity of these tumors to critical skull base structures. Methods: This is a retrospective review of 13 cases of chordomas and 2 cases of chondroid chondrosarcomas of the skull based treated with linear accelerator stereotactic radiotherapy (SRT, n = 10) or stereotactic radiosurgery (SRS, n = 5). The average time to the most recent follow-up visit was 4.5 years. The tumor characteristics, treatment details, and outcomes were recorded. Each radiation plan was reviewed, and the dosage received by the brainstem, optic apparatus, and pituitary was calculated. Results: Of the 10 patients treated with SRT, 6 were found to have unchanged or decreased tumor size as determined from radiographic follow-up. Of the 5 patients treated with SRS, 3 were found to have stable or unchanged tumors at follow-up. The complications included 1 SRT patient who developed endocrinopathy, 2 patients (1 treated with SRS and the other with SRT), who developed cranial neuropathy, and 1 SRS patient who developed visual deficits. Additionally, 1 patient who received both SRS and SRT within 2 years for recurrence experienced transient medial temporal lobe radiation changes that resolved. Conclusions: Where proton beam therapy is unavailable, linear accelerator-based SRT or radiosurgery remains a safe option for adjuvant therapy of chordomas and chondrosarcomas of the skull base. The exposure of the optic apparatus, pituitary stalk, and brainstem must be considered during planning to minimize complications. If the optic apparatus is included in the 80% isodose line, it might be best to fractionate therapy. Exposure of the pituitary stalk should be kept to <30 Gy to minimize endocrine dysfunction. Brainstem exposure should be

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

    Energy Technology Data Exchange (ETDEWEB)

    Abdelwahab, I.F.; Zwass, A.; O' Leary, P.F.; Steiner, G.C.

    1986-03-01

    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).

  19. Degenerative Pannus Mimicking Clival Chordoma Resected via an Endoscopic Transnasal Approach.

    Science.gov (United States)

    Khaldi, Ahmad; Griauzde, Julius; Duckworth, Edward A M

    2011-05-01

    Lesions of the lower clivus represent a technically challenging subset of skull base disease that requires careful treatment. A 75-year-old woman with tongue atrophy was referred for resection of a presumed clival chordoma. The lesion was resected via an endoscopic transnasal transclival approach with no complications. Pathology revealed only chronic inflammatory tissue consistent with a degenerative pannus. Degenerative pannus should be included in the differential diagnosis of lower clival extradural lesions. The endoscopic transnasal transclival corridor should be considered for resection of such lesions as an alternative to larger, more morbid, traditional skull base approaches.

  20. 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

    with an intracranial chordoma were treated at the Karolinska University Hospital, Stockholm, Sweden. Sixteen of 22 were treated with Gamma Knife radiosurgery (GKRS) for tumour residual or progression during the disease course. Seven of 22 received adjuvant fractionated radiotherapy and 5 of these also received proton...

  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. 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.

  3. Natural history and surgical treatment of chordoma: a retrospective cohort study

    Directory of Open Access Journals (Sweden)

    Samuel Aguiar Júnior

    Full Text Available CONTEXT AND OBJECTIVE: Chordoma is a rare tumor with a high risk of locoregional recurrences. The aim of this study was analyze the long-term results from treating this pathological condition.DESIGN AND SETTING: Cohort study in a single hospital in São Paulo, Brazil.METHODS: This was a retrospective cohort study on 42 patients with chordoma who were treated at Hospital A. C. Camargo between 1980 and 2006. The hospital records were reviewed and a descriptive analysis was performed on the clinical-pathological variables. Survival curves were estimated using the Kaplan-Meier method and these were compared using the log-rank test.RESULTS: Nineteen patients were men and 23 were women. Twenty-five tumors (59.5% were located in the sacrum, eleven (26.2% in the skull base and six (14.3% in the mobile spine. Surgery was performed on 28 patients (66.7%. The resection was considered to have negative margins in 14 cases and positive margins in 14 cases. The five-year overall survival (OS was 45.4%. For surgical patients, the five-year OS was 64.3% (82.2% for negative margins and 51.9% for positive margins. In the inoperable group, OS was 37.7% at 24 months and 0% at five years.CONCLUSION: Complete resection is related to local control and definitively has a positive impact on long-term survival.

  4. Sellar Chordoma Presenting as Pseudo-macroprolactinoma with Unilateral Third Cranial Nerve Palsy

    Institute of Scientific and Technical Information of China (English)

    Hai-feng Wang; Hong-xi Ma; Cheng-yuan Ma; Yi-nan Luo; Peng-fei Ge

    2012-01-01

    We described a 61-year-old female with a sellar chordoma,which presented as pseudo-macroprolactinoma with unilateral third cranial nerve palsy.Physical examination revealed that her right upper lid could not be raised by itself,right eyeball movement limited to the abduction direction,right pupil dilated to 4.5 mm with negative reaction to light,and hemianopsia in bitemporal sides.CT scanning showed a hyperdense lesion at sellar region without bone destruction.Magnetic resonance imaging (MRI) revealed the tumor was 2.3 cm×1.8 cm×2.6 cm,with iso-intensity on T1WI,hyper-intensity on T2WI and heterogeneous enhancement on contrast imaging.Endocrine examination showed her serum prolactin level increased to 1,031.49 mlU/ml.The tumor was sub-totally resected via pterional craniotomy under microscope and was histologically proven to be a chordoma.Postoperatively,she recovered uneventfully but ptosis and hemianopsia remained at the 6th month.

  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. First histologically confirmed case of a classic chordoma arising in a precursor benign notochordal lesion: differential diagnosis of benign and malignant notochordal lesions

    International Nuclear Information System (INIS)

    Yamaguchi, Takehiko; Yamato, Minoru; Saotome, Koichi

    2002-01-01

    The first histologically confirmed case of a classic chordoma arising in a precursor benign notochordal lesion is presented and the differential diagnosis between benign and malignant notochordal lesions is discussed. A 57-year-old man presented with a classic chordoma in the coccyx. The resected specimen demonstrated a small intraosseous benign notochordal lesion in the coccyx, which was adjacent to the classic chordoma. Also seen were two separate, similar benign lesions in the sacrum. The classic chordoma consisted of multiple lobules that were separated by thin fibrous septa and that showed cords or strands of atypical physaliphorous cells set within an abundant myxoid matrix. In contrast, the benign lesions consisted of intraosseous sheets of bland physaliphorous cells without any extracellular matrix. The affected bone trabeculae showed sclerotic reactions. It was concluded that benign and malignant notochordal lesions can be distinguished microscopically. (orig.)

  7. 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.

  8. 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

  9. Radioresistance of chordoma cells is associated with the ATM/ATR pathway, in which RAD51 serves as an important downstream effector.

    Science.gov (United States)

    Zhang, Chao; Wang, Bing; Li, Lei; Li, Yawei; Li, Pengzhi; Lv, Guohua

    2017-09-01

    Surgery followed by radiotherapy is the standard treatment for chordomas, which are a rare but low-grade type of bone cancer arising from remnants of the embryonic notochord. However, disease recurrence following radiotherapy is common, most likely due to endogenous DNA repair mechanisms that promote cell survival upon radiation strikes. The ataxia telangiectasia mutated/ataxia telangiectasia mutated and Rad3 related (ATM/ATR)-mediated pathway has a critical role in DNA repair mechanisms; however, it has rarely been investigated in chordomas. In the present study, the expression of signal molecules related to the ATM/ATR pathway in chordoma tissues and adjacent normal tissues were initially examined using immunohistochemistry and western blot analysis. Chordoma U-CH1 and U-CH2 cells were subsequently used to investigate cell responses to ionizing radiation and the potential protective actions mediated by the ATM/ATR pathway. Phosphorylated (p)-ATM, p-ATR, γ-H2A histone family, member X (H2AX) and RAD51 were significantly upregulated in chordoma tissues relative to adjacent normal tissues (PATM, γ-H2AX and RAD51 expression in U-CH1 cells (PATM, p-ATR and RAD51 levels in U-CH2 cells (PATM/ATR pathway, in which RAD51 serves as an important downstream effector. Thus, RAD51 presents a promising therapeutic target for improving the outcome of radiotherapy treatment in chordomas.

  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. 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

  12. 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

  13. Solitary intracranial plasmacytoma located in the spheno-clival region mimicking chordoma: a case report.

    Science.gov (United States)

    Liu, Z Y; Qi, X Q; Wu, X J; Luo, C; Lu, Y C

    2010-01-01

    Solitary intracranial plasmacytoma (SIP) is very rare. This case report presents serial findings of SIP located in the spheno-clival region in a 54-year old female who presented with an inferior hemianopia in the right eye and an enlarged physiological blind spot in both eyes. Based on the initial diagnosis of a spheno-clival region chordoma, the tumour was partially resected by the nasal-sphenoidal sinus approach. Subsequently, the correct diagnosis of SIP was made based on the pathology and immunohistochemical staining of the tumour. The patient was treated using a whole skull-base radiation therapy protocol with 45 Gy and she was in good physical condition during the subsequent 22 months. The findings of a series of similar case reports documenting SIP in 20 cases published from 1976 to 2008 are also reviewed. Based on these case reports, the key features of SIP, including their clinical manifestations, clinical imaging characteristics, treatment and prognosis, are described.

  14. The Unresolved Case of Sacral Chordoma: From Misdiagnosis to Challenging Surgery and Medical Therapy Resistance

    Science.gov (United States)

    Garofalo, Fabio; Christoforidis, Dimitrios; di Summa, Pietro G.; Gay, Béatrice; Cherix, Stéphane; Raffoul, Wassim; Matter, Maurice

    2014-01-01

    Purpose A sacral chordoma is a rare, slow-growing, primary bone tumor, arising from embryonic notochordal remnants. Radical surgery is the only hope for cure. The aim of our present study is to analyse our experience with the challenging treatment of this rare tumor, to review current treatment modalities and to assess the outcome based on R status. Methods Eight patients were treated in our institution between 2001 and 2011. All patients were discussed by a multidisciplinary tumor board, and an en bloc surgical resection by posterior perineal access only or by combined anterior/posterior accesses was planned based on tumor extension. Results Seven patients underwent radical surgery, and one was treated by using local cryotherapy alone due to low performance status. Three misdiagnosed patients had primary surgery at another hospital with R1 margins. Reresection margins in our institution were R1 in two and R0 in one, and all three recurred. Four patients were primarily operated on at our institution and had en bloc surgery with R0 resection margins. One had local recurrence after 18 months. The overall morbidity rate was 86% (6/7 patients) and was mostly related to the perineal wound. Overall, 3 out of 7 resected patients were disease-free at a median follow-up of 2.9 years (range, 1.6-8.0 years). Conclusion Our experience confirms the importance of early correct diagnosis and of an R0 resection for a sacral chordoma invading pelvic structures. It is a rare disease that requires a challenging multidisciplinary treatment, which should ideally be performed in a tertiary referral center. PMID:24999463

  15. Carbon Ion Radiation Therapy for Unresectable Sacral Chordoma: An Analysis of 188 Cases

    Energy Technology Data Exchange (ETDEWEB)

    Imai, Reiko, E-mail: r_imai@nirs.go.jp [Research Center Hospital for Charged Particle Therapy, National Institute of Radiological Sciences, Chiba (Japan); Kamada, Tadashi [Research Center Hospital for Charged Particle Therapy, National Institute of Radiological Sciences, Chiba (Japan); Araki, Nobuhito [Department of Orthopedic Surgery, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka (Japan); Abe, Satoshi; Iwamoto, Yukihide; Ozaki, Toshifumi; Kanehira, Chihiro; Kaya, Mitsunori; Takahashi, Kazuhisa; Chuman, Hirokazu; Tsujii, Hirohiko; Tsuneyoshi, Masazumi; Nishida, Yoshihiro; Hiraga, Hiroaki; Hiruma, Toru; Machinami, Rikuo; Matsumine, Akihiko; Matsumoto, Seiichi; Morioka, Hideo; Yamaguchi, Takehiko; and others

    2016-05-01

    Purpose: To evaluate the results of carbon ion radiation therapy administered to 188 patients with unresectable primary sacral chordomas. Patients and Methods: One hundred eighty-eight patients were treated with carbon ion radiation therapy at a single institute between 1996 and 2013 and retrospectively analyzed. The median age was 66 years. The highest proximal invasion reached past S2 level in 137 patients. The median clinical target volume was 345 cm{sup 3}. One hundred six patients received 67.2 gray equivalents (GyE)/16 fractions (fr), 74 patients received 70.4 GyE/16 fr, 7 patients received 73.6 GyE/16 fr, and 1 patient received 64.0 GyE/16 fr. Results: The median follow-up period was 62 months (range, 6.8-147.5 months). Seventy percent of patients were followed for 5 years or until death. The 5-year local control, overall survival, and disease-free survival rates were 77.2%, 81.1%, and 50.3%, respectively. Forty-one patients had a local recurrence. Sex, tumor volume, level of proximal invasion, and irradiated dose were unrelated to local control. There was grade 3 toxicity of the peripheral nerves in 6 patients and grade 4 toxicity of the skin in 2 patients. Ambulation remained in 97% of patients. Conclusions: Carbon ion radiation therapy was safe and effective for unresectable chordoma and provided good local control and survival while preserving ambulation.

  16. Clinical Outcome of Sacral Chordoma With Carbon Ion Radiotherapy Compared With Surgery

    International Nuclear Information System (INIS)

    Nishida, Yoshihiro; Kamada, Tadashi; Imai, Reiko; Tsukushi, Satoshi; Yamada, Yoshihisa; Sugiura, Hideshi; Shido, Yoji; Wasa, Junji; Ishiguro, Naoki

    2011-01-01

    Purpose: To evaluate the efficacy, post-treatment function, toxicity, and complications of carbon ion radiotherapy (RT) for sacral chordoma compared with surgery. Methods and Materials: The records of 17 primary sacral chordoma patients treated since 1990 with surgery (n = 10) or carbon ion RT (n = 7) were retrospectively analyzed for disease-specific survival, local recurrence-free survival, complications, and functional outcome. The applied carbon ion dose ranged from 54.0 Gray equivalent (GyE) to 73.6 GyE (median 70.4). Results: The mean age at treatment was 55 years for the surgery group and 65 years for the carbon ion RT group. The median duration of follow-up was 76 months for the surgery group and 49 months for the carbon ion RT group. The local recurrence-free survival rate at 5 years was 62.5% for the surgery and 100% for the carbon ion RT group, and the disease-specific survival rate at 5 years was 85.7% and 53.3%, respectively. Urinary-anorectal function worsened in 6 patients (60%) in the surgery group, but it was unchanged in all the patients who had undergone carbon ion RT. Postoperative wound complications requiring reoperation occurred in 3 patients (30%) after surgery and in 1 patient (14%) after carbon ion RT. The functional outcome evaluated using the Musculoskeletal Tumor Society scoring system revealed 55% in the surgery group and 75% in the carbon ion RT group. Of the six factors in this scoring system, the carbon ion RT group had significantly greater scores in emotional acceptance than did the surgery group. Conclusion: Carbon ion RT results in a high local control rate and preservation of urinary-anorectal function compared with surgery.

  17. Effectiveness and Safety of Spot Scanning Proton Radiation Therapy for Chordomas and Chondrosarcomas of the Skull Base: First Long-Term Report

    International Nuclear Information System (INIS)

    Ares, Carmen; Hug, Eugen B.; Lomax, Antony J.; Bolsi, Alessandra; Timmermann, Beate; Rutz, Hans Peter; Schuller, Jan C.; Pedroni, Eros; Goitein, Gudrun

    2009-01-01

    Purpose: To evaluate effectiveness and safety of spot-scanning-based proton radiotherapy (PT) in skull-base chordomas and chondrosarcomas. Methods and Materials: Between October 1998 and November 2005, 64 patients with skull-base chordomas (n = 42) and chondrosarcomas (n = 22) were treated at Paul Scherrer Institute with PT using spot-scanning technique. Median total dose for chordomas was 73.5 Gy(RBE) and 68.4 Gy(RBE) for chondrosarcomas at 1.8-2.0 Gy(RBE) dose per fraction. Local control (LC), disease specific survival (DSS), and overall survival (OS) rates were calculated. Toxicity was assessed according to CTCAE, v. 3.0. Results: Mean follow-up period was 38 months (range, 14-92 months). Five patients with chordoma and one patient with chondrosarcoma experienced local recurrence. Actuarial 5-year LC rates were 81% for chordomas and 94% for chondrosarcomas. Brainstem compression at the time of PT (p = 0.007) and gross tumor volume >25 mL (p = 0.03) were associated with lower LC rates. Five years rates of DSS and OS were 81% and 62% for chordomas and 100% and 91% for chondrosarcomas, respectively. High-grade late toxicity consisted of one patient with Grade 3 and one patient with Grade 4 unilateral optic neuropathy, and two patients with Grade 3 central nervous system necrosis. No patient experienced brainstem toxicity. Actuarial 5-year freedom from high-grade toxicity was 94%. Conclusions: Our data indicate safety and efficacy of spot-scanning based PT for skull-base chordomas and chondrosarcomas. With target definition, dose prescription and normal organ tolerance levels similar to passive-scattering based PT series, complication-free, tumor control and survival rates are at present comparable.

  18. Is there a role for conventional MRI and MR diffusion-weighted imaging for distinction of skull base chordoma and chondrosarcoma?

    Science.gov (United States)

    Müller, Uta; Kubik-Huch, Rahel A; Ares, Carmen; Hug, Eugen B; Löw, Roland; Valavanis, Antonios; Ahlhelm, Frank J

    2016-02-01

    Chordoma and chondrosarcoma are locally invasive skull base tumors with similar clinical symptoms and anatomic imaging features as reported in the literature. To determine differentiation of chordoma and chondrosarcoma of the skull base with conventional magnetic resonance imaging (cMRI) and diffusion-weighted MR imaging (DWI) in comparison to histopathological diagnosis. This retrospective study comprised 96 (chordoma, n = 64; chondrosarcoma, n = 32) patients with skull base tumors referred to the Paul Scherrer Institute (PSI) for proton therapy. cMRI signal intensities of all tumors were investigated. In addition, median apparent diffusion coefficient (ADC) values were measured in a subgroup of 19 patients (chordoma, n = 11; chondrosarcoma, n = 8). The majority 81.2% (26/32) of chondrosarcomas displayed an off-midline growth pattern, 18.8% (6/32) showed clival invasion, 18.8% (6/32) were located more centrally. Only 4.7% (3/64) of chordomas revealed a lateral clival origin. Using cMRI no significant differences in MR signal intensities were observed in contrast to significantly different ADC values (subgroup of 19/96 patients examined by DWI), with the highest mean value of 2017.2 × 10(-6 )mm(2)/s (SD, 139.9( )mm(2)/s) for chondrosarcoma and significantly lower value of 1263.5 × 10(-6 )mm(2)/s (SD, 100.2 × 10(-6 )mm(2)/s) for chordoma (P = 0.001/median test). An off-midline growth pattern can differentiate chondrosarcoma from chordoma on cMRI in a majority of patients. Additional DWI is a promising tool for the differentiation of these skull base tumors. © The Foundation Acta Radiologica 2015.

  19. 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.)

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

    International Nuclear Information System (INIS)

    Gong, Tao; Wang, Guangbin; Gao, Fei; Chen, Xin; Liu, Yubo; Yang, Li; Chen, Weibo

    2016-01-01

    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.)

  1. 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.)

  2. Cervical Intradural Disc Herniation Causing Progressive Quadriparesis After Spinal Manipulation Therapy: A Case Report and Literature Review.

    Science.gov (United States)

    Yang, Hwan-Seo; Oh, Young-Min; Eun, Jong-Pil

    2016-02-01

    Cervical intradural disc herniation (IDH) is an extremely rare condition, comprising only 0.27% of all disc herniations. Three percent of IDHs occur in the cervical, 5% in the thoracic, and over 92% in the lumbar spinal canal. There have been a total of 31 cervical IDHs reported in the literature. The pathogenesis and imaging characteristics of IDH are not fully understood. A preoperative diagnosis is key to facilitating prompt intradural exploration in patients with ambivalent findings, as well as in preventing reoperation. The purpose of reporting our case is to remind clinicians to consider the possibility of cervical IDH during spinal manipulation therapy in patient with chronic neck pain.The patient signed informed consent for publication of this case report and any accompanying image. The ethical approval of this study was waived by the ethics committee of Chonbuk National University Hospital, because this study was case report and the number of patients was manipulation for chronic neck pain over the course of a month. The day prior, he had noticed neck pain and tingling in the bilateral upper and lower extremities during the manipulation procedure. The following day, he presented with bilateral weakness of all 4 extremities, which rendered him unable to walk. Neurological examination demonstrated a positive Hoffmann sign and ankle clonus bilaterally, hypoesthesia below the C5 dermatome, 3/5 strength in the bilateral upper extremities, and 2/5 strength in the lower extremities. This motor weakness was progressive, and he further complained of voiding difficulty.Urgent magnetic resonance imaging (MRI) of the cervical spine revealed large, central disc herniations at C4-C5 and C5-C6 that caused severe spinal cord compression and surrounding edema. We performed C4-C5-C6 anterior cervical discectomy and fusion.The patient's limb weakness improved rapidly within 1 day postoperatively, and he was discharged 4 weeks later. At his 12-month follow-up, the patient had

  3. Active raster scanning with carbon ions. Reirradiation in patients with recurrent skull base chordomas and chondrosarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Uhl, Matthias; Welzel, Thomas; Oelmann, Jan; Habl, Gregor; Hauswald, Henrik; Jensen, Alexandra; Debus, Juergen; Herfarth, Klaus [University of Heidelberg, Department of Radiation Oncology, Heidelberg (Germany); Ellerbrock, Malte [Heidelberg Ion Therapy Center (HIT), Heidelberg (Germany)

    2014-07-15

    To evaluate the safety and efficacy of reirradiation with carbon ions in patients with relapse of skull base chordoma and chondrosarcoma. Reirradiation with carbon ions was performed on 25 patients with locally recurrent skull base chordoma (n = 20) or chondrosarcoma (n = 5). The median time between the last radiation exposure and the reirradiation with carbon ions was 7 years. In the past, 23 patients had been irradiated once, two patients twice. Reirradiation was delivered using the active raster scanning method. The total median dose was 51.0 GyE carbon ions in a weekly regimen of five to six fractions of 3 GyE. Local progression-free survival (LPFS) was evaluated using the Kaplan-Meier method; toxicity was evaluated using the NCI Common Terminology Criteria for Adverse Events (CTCAE v.4.03). The treatment could be finished in all patients without interruption. In 80 % of patients, symptom control was achieved after therapy. The 2-year-LPFS probability was 79.3 %. A PTV volume of < 100 ml or a total dose of > 51 GyE was associated with a superior local control rate. The therapy was associated with low acute toxicity. One patient developed grade 2 mucositis during therapy. Furthermore, 12 % of patients had tympanic effusion with mild hypacusis (grade 2), while 20 % developed an asymptomatic temporal lobe reaction after treatment (grade 1). Only one patient showed a grade 3 osteoradionecrosis. Reirradiation with carbon ions is a safe and effective method in patients with relapsed chordoma and chondrosarcoma of the skull base. (orig.) [German] Evaluierung der Sicherheit und Wirksamkeit einer Re-Bestrahlung mittels Kohlenstoffionen bei Patienten mit Lokalrezidiv eines Chordoms und Chondrosarkoms der Schaedelbasis. Bei 25 Patienten mit einem Lokalrezidiv eines Chordoms (n = 20) oder Chondrosarkoms (n = 5) der Schaedelbasis erfolgte eine Re-Bestrahlung mittels Kohlenstoffionen. Die mediane Zeit zwischen letzter Bestrahlung und Re-Bestrahlung mit Kohlenstoffionen

  4. 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.

  5. A potential therapy for chordoma via antibody-dependent cell-mediated cytotoxicity employing NK or high-affinity NK cells in combination with cetuximab.

    Science.gov (United States)

    Fujii, Rika; Schlom, Jeffrey; Hodge, James W

    2018-05-01

    OBJECTIVE Chordoma is a rare bone tumor derived from the notochord and is resistant to conventional therapies such as chemotherapy, radiotherapy, and targeting therapeutics. Expression of epidermal growth factor receptor (EGFR) in a large proportion of chordoma specimens indicates a potential target for therapeutic intervention. In this study the authors investigated the potential role of the anti-EGFR antibody cetuximab in immunotherapy for chordoma. METHODS Since cetuximab is a monoclonal antibody of the IgG1 isotype, it has the potential to mediate antibody-dependent cell-mediated cytotoxicity (ADCC) employing natural killer (NK) cells as effectors. Polymorphisms in the CD16 allele expressed on NK cells have been shown to influence the degree of ADCC of tumor cells, with the high-affinity valine (V)/V allele being responsible for more lysis than the V/phenylalanine (F) or FF allele. Unfortunately, however, only approximately 10% of the population expresses the VV allele on NK cells. An NK cell line, NK-92, has now been engineered to endogenously express IL-2 and the high-affinity CD16 allele. These irradiated high-affinity (ha)NK cells were analyzed for lysis of chordoma cells with and without cetuximab, and the levels of lysis observed in ADCC were compared with those of NK cells from donors expressing the VV, VF, and FF alleles. RESULTS Here the authors demonstrate for the first time 1) that cetuximab in combination with NK cells can mediate ADCC of chordoma cells; 2) the influence of the NK CD16 polymorphism in cetuximab-mediated ADCC for chordoma cell lysis; 3) that engineered haNK cells-that is, cells transduced to express the CD16 V158 FcγRIIIa receptor-bind cetuximab with similar affinity to normal NK cells expressing the high-affinity VV allele; and 4) that irradiated haNK cells induce ADCC with cetuximab in chordoma cells. CONCLUSIONS These studies provide rationale for the use of cetuximab in combination with irradiated haNK cells for therapy for

  6. Enhanced killing of chordoma cells by antibody-dependent cell-mediated cytotoxicity employing the novel anti-PD-L1 antibody avelumab

    OpenAIRE

    Fujii, Rika; Friedman, Eitan R.; Richards, Jacob; Tsang, Kwong Y.; Heery, Christopher R.; Schlom, Jeffrey; Hodge, James W.

    2016-01-01

    Chordoma, a rare bone tumor derived from the notochord, has been shown to be resistant to conventional therapies. Checkpoint inhibition has shown great promise in immune-mediated therapy of diverse cancers. The anti-PD-L1 mAb avelumab is unique among checkpoint inhibitors in that it is a fully human IgG1 capable of mediating antibody-dependent cell-mediated cytotoxicity (ADCC) of PD-L1-expressing tumor cells. Here, we investigated avelumab as a potential therapy for chordoma. We examined 4 ch...

  7. Radiation therapy for chordoma and chondrosarcoma of the skull base and the cervical spine. Prognostic factors and patterns of failure

    International Nuclear Information System (INIS)

    Noel, G.; Jauffret, E.; Mammar, H.; Ferrand, R.; Habrand, J.L.; Crevoisier, R. de; Haie-Meder, C.; Beaudre, A.; Dederke, S.; Hasboun, D.; Boisserie, G.; Pontvert, D.; Gaboriaud, G.; Guedea, F.; Petriz, L.; Mazeron, J.J.

    2003-01-01

    Background: Prospective analysis of local tumor control, survival and treatment complications in 67 consecutive patients treated with fractionated photon and proton radiation for chordoma or chondrosarcoma of the base of the skull and the cervical spine. Patients and Methods: Between December 1995 and January 2000, 67 patients with a median age of 52 years (range: 14-85 years), were treated at the Centre de Protontherapie d'Orsay (CPO), France, using the 201-MeV proton beam, 49 for chordoma and 18 for chondrosarcoma. Irradiation combined high-energy photons and protons. Photons represented two thirds of the total dose and protons one third. The median total dose delivered within gross tumor volume (GTV) was 67 cobalt gray equivalents (CGE; range: 60-70 CGE). Results: Within a median follow-up of 29 months (range: 4-71 months), the 3-year local control rates were 71% and 85% for chordomas and chondrosarcomas, respectively, and the 3-year overall survival rates 88% and 75%, respectively. 14 tumors (21.5%) failed locally (eight within the GTV, four within the clinical target volume [CTV], and two without further assessment). Seven patients died from their tumor and another one from a nonrelated condition (pulmonary embolism). The maximum tumor diameter and, similarly, the GTV were larger in relapsing patients, compared with the rest of the population: 56 mm vs 44 mm (p = 0.024) and 50 ml vs 22 ml (p = 0.0083), respectively. In univariate analysis, age ≤ 52 years at the time of radiotherapy (p = 0.002), maximum diameter < 45 mm (p = 0.02), and GTV < 28 ml (p = 0.02) impacted positively on local control. On multivariate analysis, only age was an independent prognostic factor of local control. Conclusion: In chordomas and chondrosarcomas of the skull base and cervical spine, combined photon and proton radiation therapy offers excellent chances of cure. In two thirds of the cases, relapses are located in the GTV. Maximum diameter, GTV, and age are prognostic indicators

  8. Differentiation of primary chordoma, giant cell tumor and schwannoma of the sacrum by CT and MRI

    Energy Technology Data Exchange (ETDEWEB)

    Si, Ming-Jue, E-mail: smjsh@hotmail.com [Department of Radiology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025 (China); Wang, Cheng-Sheng [Department of Radiology, Union Hospital, Fujian Medical University, Fuzhou 350001 (China); Ding, Xiao-Yi, E-mail: dingxiaoyi1965@hotmail.com [Department of Radiology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025 (China); Yuan, Fei, E-mail: yuanfeirj@hotmail.com [Department of Pathology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025 (China); Du, Lian-Jun; Lu, Yong [Department of Radiology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025 (China); Zhang, Wei-Bin [Department of Orthopedics, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025 (China)

    2013-12-01

    Objective: To evaluate criteria to differentiate sacral chordoma (SC), sacral giant cell tumor (SGCT) and giant sacral schwannoma (GSS) with CT and MRI. Materials and methods: CT and MR images of 22 SCs, 19 SGCTs and 8 GSSs were reviewed. The clinical and imaging features of each tumor were analyzed. Results: The mean ages of SC, SGCT and GSS were 55.1 ± 10.7, 34.3 ± 10.7 and 42.4 ± 15.7 years old. SCs (77.3%) were predominantly located in the midline of lower sacrum, while most SGCTs (73.7%) and GSSs (87.5%) were eccentrically located in upper sacrum. There were significant differences in age, location, eccentricity, morphology of bone residues, intratumoral bleeding and septations. Multiple small cysts were mainly observed in SGCTs (73.7%) with large central cysts in GSSs (87.5%). SGCTs expanded mainly inside sacrum while SCs and GSSs often extended into pelvic cavity (P = 0.0022). Involvement of sacroiliac joints and muscles were also different. Ascending extension within sacral canal was only displayed in SCs. The preservation of intervertebral discs showed difference between large and small tumors (P = 0.0002), regardless of tumor type (P = 0.095). No significant difference was displayed in gender (P = 0.234) or tumor size (P = 0.0832) among three groups. Conclusion: Age, epicenter of the lesion (midline vs. eccentric and upper vs. lower sacral vertebra), bone residues, cysts, bleeding, septation, expanding pattern, muscles and sacroiliac joint involvement can be criteria for diagnosis. Fluid–fluid level is specific for SGCTs and ascending extension within the sacral canal for SCs. The preservation of intervertebral discs is related to tumor size rather than tumor type.

  9. Sacral chordomas: Impact of high-dose proton/photon-beam radiation therapy combined with or without surgery for primary versus recurrent tumor

    International Nuclear Information System (INIS)

    Park, Lily; De Laney, Thomas F.; Liebsch, Norbert J.; Hornicek, Francis J.; Goldberg, Saveli; Mankin, Henry; Rosenberg, Andrew E.; Rosenthal, Daniel I.; Suit, Herman D.

    2006-01-01

    Purpose: To assess the efficacy of definitive treatment of sacral chordoma by high-dose proton/photon-beam radiation therapy alone or combined with surgery. Methods and Materials: The records of 16 primary and 11 recurrent sacral chordoma patients treated from November 1982 to November 2002 by proton/photon radiation therapy alone (6 patients) or combined with surgery (21 patients) have been analyzed for local control, survival, and treatment-related morbidity. The outcome analysis is based on follow-up information as of 2005. Results: Outcome results show a large difference in local failure rate between patients treated for primary and recurrent chordomas. Local control results by surgery and radiation were 12/14 vs. 1/7 for primary and recurrent lesions. For margin-positive patients, local control results were 10 of 11 and 0 of 5 in the primary and recurrent groups, respectively; the mean follow-up on these locally controlled patients was 8.8 years (4 at 10.3, 12.8, 17, and 21 years). Radiation alone was used in 6 patients, 4 of whom received ≥73.0 Gy (E); local control was observed in 3 of these 4 patients for 2.9, 4.9, and 7.6 years. Conclusion: These data indicate a high local control rate for surgical and radiation treatment of primary (12 of 14) as distinct from recurrent (1 of 7) sacral chordomas. Three of 4 chordomas treated by ≥73.0 Gy (E) of radiation alone had local control; 1 is at 91 months. This indicates that high-dose proton/photon therapy offers an effective treatment option

  10. [Usefulness of neuroendoscopy and a neuronavigator for removal of clival chordoma].

    Science.gov (United States)

    Miyagi, A; Maeda, K; Sugawara, T

    1998-02-01

    We report a case of large clival chordoma. The patient was a 56-year-old male who was admitted to our hospital with left eye ptosis and diplopia of 2 months duration. On admission, neurological examinations revealed oculomotor nerve palsy of the left eye. Skull radiographs with polytomographs demonstrated marked destruction of the clivus. A plain computed tomography (CT) scan revealed a large iso-attenuated mass in the clivus, extending anteriorly into the sphenoidal sinus, superiorly into the suprasellar cistern, bilaterally into the petrous apex, posteriorly into the prepontine cistern and caudally into the foramen magnum. An enhanced CT scan demonstrated a slightly enhanced tumor. A high-resolution bone-window CT scan revealed marked destruction of the clivus and bilateral petrous apex. Magnetic resonance imaging (MRI) scans disclosed a large enhanced mass extending superiorly into the suprasellar cistern, bilaterally into the petrous apex and inferiorly into the foramen magnum. The tumor extended so widely that we decided on a one-stage operation via a transsphenoidal sublabial transseptal approach and transoral transpalatal approach. At surgery, we employed a neuronavigator and Codman 4-mm rigid neuroendoscope with 0 degree, 30 degrees and 70 degrees angled lenses. The tumor was very soft and suckable, and could be easily removed by applying CUSA, a pituitary curette and suction. The neuronavigator was particularly useful because the surgeon had a real-time two-dimensional representation of the position of the tip of this device in the corresponding imaging space intraoperatively. The neuroendoscope also proved useful, since remnant tumor tissues that could not be seen under an operating microscope were frequently recognized near or around the entrance of the tumor cavity, cavernous sinus region and petroclival junction area. The surgeon was able to remove these remnants safely by checking on the neuroendoscope monitor. The tumor was excised completely. The

  11. 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

  12. Assessment of outcome in patients undergoing surgery for intradural spinal tumor using the multidimensional patient-rated Core Outcome Measures Index and the modified McCormick Scale.

    Science.gov (United States)

    Bellut, David; Burkhardt, Jan-Karl; Mannion, Anne F; Porchet, François

    2015-08-01

    OBJECT The aim of this study was to evaluate outcome in patients undergoing surgical treatment for intradural spinal tumor using a patient-oriented, self-rated, outcome instrument and a physician-based disease-specific instrument. METHODS Prospectively collected data from 63 patients with intradural spinal tumor were analyzed in relation to scores on the multidimensional patient-rated Core Outcome Measures Index (COMI) and the physician-rated modified McCormick Scale, before and at 3 and 12 months after surgery. RESULTS There was no statistically significant difference between the scores on the modified McCormick Scale preoperatively and at the 3-month follow-up, though there was a trend for improvement (p = 0.073); however, comparisons between the scores determined preoperatively and at the 12-month follow-up, as well as 3- versus 12-month follow-ups, showed a statistically significant improvement in each case (p 0.05) up to 12 months postoperatively. In contrast, the overall COMI score, "worst pain," quality of life, and social disability not only showed a significant reduction from before surgery to 3 months after surgery (p 0.05), but did show a significant improvement (p = 0.011) from 3 months to 12 months after surgery. At the 3- and 12-month follow-ups, 85.2% and 83.9% of patients, respectively, declared that the surgical procedure had helped/helped a lot; 95.1% and 95.2%, respectively, declared that they were satisfied/very satisfied with their care. CONCLUSIONS COMI is a feasible tool to use in the evaluation of baseline symptoms and outcome in patients undergoing surgery for intradural spinal tumor. COMI was able to detect changes in outcome at 3 months after surgery (before changes were apparent on the modified McCormick Scale) and on later postoperative follow-up. The COMI subdomains are valuable for monitoring the patient's reintegration into society and the work environment. The addition of an item that specifically covers neurological deficits may

  13. Anterolateral meningioma of the foramen magnum and high cervical spine presenting intradural and extradural growth in a child: case report and literature review.

    Science.gov (United States)

    Athanasiou, Alkinoos; Magras, Ioannis; Sarlis, Panagiotis; Spyridopoulos, Evangelos; Polyzoidis, Konstantinos

    2015-12-01

    We report a rare case of anterolateral meningioma of the foramen magnum (FMM) and high cervical spine presenting both intradural and extradural growth in a 7.5-year-old boy. We also performed a review of the relevant peer-reviewed literature. The patient presented with progressive tetraparesis and gait instability. Neuroimaging revealed an anterolateral tumor of the foramen magnum, C1 and C2 cervical spine level. The patient was treated in two stages: During the first operation, the extradural part was resected while the intradural part was removed in a second operation. Following the second operation, the patient showed almost complete neurological recovery as a result of cervical spinal cord and brainstem decompression but was complicated with cerebrospinal fluid leakage and infection by Acinetobacter. He sustained two further operations for dural sealing and external ventricular drainage and was treated with intraventricular administration of antibiotics. Histopathology of the tumor confirmed a meningotheliomatous meningioma. At the 6-month post-op follow-up examination, the patient exhibited complete neurological recovery and no radiological tumor recurrence. To the authors' best knowledge, we report the third case of sporadic pediatric meningioma of the foramen magnum and high cervical compartments with an extradural growth. Accurate pre-operative estimation of possible extradural growth is crucial towards surgical planning and sufficient treatment. Treatment of choice is total resection in a single operating session to avoid re-operations and increased risk of complications. If not possible, a re-operation should always attempt to secure the desired result.

  14. Combination of photon and proton radiation therapy for chordomas and chondrosarcomas of the skull base: the Centre de Protontherapie D'Orsay experience

    International Nuclear Information System (INIS)

    Noeel, Georges; Habrand, Jean-Louis; Mammar, Hamid; Pontvert, Dominique; Haie-Meder, Christine; Hasboun, Dominique; Moisson, Patricia; Ferrand, Regis; Beaudre, Anne; Boisserie, Gilbert; Gaboriaud, Genevieve; Mazal, Alexandre; Kerody, Katia; Schlienger, Michel; Mazeron, Jean-Jacques

    2001-01-01

    Purpose: Prospective analysis of local tumor control, survival, and treatment complications in 44 consecutive patients treated with fractionated photon and proton radiation for a chordoma or chondrosarcoma of the skull base. Methods and Materials : Between December 1995 and December 1998, 45 patients with a median age of 55 years (14-85) were treated using a 201-MeV proton beam at the Centre de Protontherapie d'Orsay, 34 for a chordoma and 11 for a chondrosarcoma. Irradiation combined high-energy photons and protons. Photons represented two-thirds of the total dose and protons one-third. The median total dose delivered within the gross tumor volume was 67 cobalt Gray equivalent (CGE) (range: 60-70). Results: With a mean follow-up of 30.5 months (range: 2-56), the 3-year local control rates for chordomas and chondrosarcomas were 83.1% and 90%, respectively, and 3-year overall survival rates were 91% and 90%, respectively. Eight patients (18%) failed locally (7 within the clinical tumor volume and 1 unknown). Four patients died of tumor and 2 others of intercurrent disease. In univariate analysis, young age at time of radiotherapy influenced local control positively (p < 0.03), but not in multivariate analysis. Only 2 patients presented Grade 3 or 4 complications. Conclusion: In skull-base chordomas and chondrosarcomas, the combination of photons with a proton boost of one-third the total dose offers an excellent chance of cure at the price of an acceptable toxicity. These results should be confirmed with a longer follow-up

  15. Positron Emission Tomography/Computed Tomography Imaging of Residual Skull Base Chordoma Before Radiotherapy Using Fluoromisonidazole and Fluorodeoxyglucose: Potential Consequences for Dose Painting

    Energy Technology Data Exchange (ETDEWEB)

    Mammar, Hamid, E-mail: hamid.mammar@unice.fr [Radiation Oncology Department, Antoine Lacassagne Center, Nice (France); CNRS-UMR 6543, Institute of Developmental Biology and Cancer, University of Nice Sophia Antipolis, Nice (France); Kerrou, Khaldoun; Nataf, Valerie [Department of Nuclear Medicine and Radiopharmacy, Tenon Hospital, and University Pierre et Marie Curie, Paris (France); Pontvert, Dominique [Proton Therapy Center of Orsay, Curie Institute, Paris (France); Clemenceau, Stephane [Department of Neurosurgery, Pitie-Salpetriere Hospital, Paris (France); Lot, Guillaume [Department of Neurosurgery, Adolph De Rothschild Foundation, Paris (France); George, Bernard [Department of Neurosurgery, Lariboisiere Hospital, Paris (France); Polivka, Marc [Department of Pathology, Lariboisiere Hospital, Paris (France); Mokhtari, Karima [Department of Pathology, Pitie-Salpetriere Hospital, Paris (France); Ferrand, Regis; Feuvret, Loiec; Habrand, Jean-louis [Proton Therapy Center of Orsay, Curie Institute, Paris (France); Pouyssegur, Jacques; Mazure, Nathalie [CNRS-UMR 6543, Institute of Developmental Biology and Cancer, University of Nice Sophia Antipolis, Nice (France); Talbot, Jean-Noeel [Department of Nuclear Medicine and Radiopharmacy, Tenon Hospital, and University Pierre et Marie Curie, Paris (France)

    2012-11-01

    Purpose: To detect the presence of hypoxic tissue, which is known to increase the radioresistant phenotype, by its uptake of fluoromisonidazole (18F) (FMISO) using hybrid positron emission tomography/computed tomography (PET/CT) imaging, and to compare it with the glucose-avid tumor tissue imaged with fluorodeoxyglucose (18F) (FDG), in residual postsurgical skull base chordoma scheduled for radiotherapy. Patients and Methods: Seven patients with incompletely resected skull base chordomas were planned for high-dose radiotherapy (dose {>=}70 Gy). All 7 patients underwent FDG and FMISO PET/CT. Images were analyzed qualitatively by visual examination and semiquantitatively by computing the ratio of the maximal standardized uptake value (SUVmax) of the tumor and cerebellum (T/C R), with delineation of lesions on conventional imaging. Results: Of the eight lesion sites imaged with FDG PET/CT, only one was visible, whereas seven of nine lesions were visible on FMISO PET/CT. The median SUVmax in the tumor area was 2.8 g/mL (minimum 2.1; maximum 3.5) for FDG and 0.83 g/mL (minimum 0.3; maximum 1.2) for FMISO. The T/C R values ranged between 0.30 and 0.63 for FDG (median, 0.41) and between 0.75 and 2.20 for FMISO (median,1.59). FMISO T/C R >1 in six lesions suggested the presence of hypoxic tissue. There was no correlation between FMISO and FDG uptake in individual chordomas (r = 0.18, p = 0.7). Conclusion: FMISO PET/CT enables imaging of the hypoxic component in residual chordomas. In the future, it could help to better define boosted volumes for irradiation and to overcome the radioresistance of these lesions. No relationship was founded between hypoxia and glucose metabolism in these tumors after initial surgery.

  16. Isolated Petroclival Craniopharyngioma with Aggressive Skull Base Destruction

    Science.gov (United States)

    Lee, Young-Hen; Lim, Dong-Jun; Park, Jung-Yul; Chung, Yong-Gu; Kim, Young-Sik

    2009-01-01

    We report a rare case of petroclival craniopharyngioma with no connection to the sellar or suprasellar region. MRI and CT images revealed a homogenously enhancing retroclival solid mass with aggressive skull base destruction, mimicking chordoma or aggressive sarcoma. However, there was no calcification or cystic change found in the mass. Here, we report the clinical features and radiographic investigation of this uncommon craniopharyngioma arising primarily in the petroclival region. PMID:19881982

  17. Radiation Therapy of a Chordoma of the Thoracic Vertebra-a Case Report and Review of Literatures-

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Joo Young; Choi, Myung Sun [Korea University College of Medicine, Seoul (Korea, Republic of)

    1988-12-15

    Chordom is a malignant tumor arising from the primitive notochord involving the axial skeleton. It usually occurs at sacrococcygeal and basisphenoidal area but only rarely does at other vertebral areas, especially at the thoracic vertebrae. It has a slow growth rate and is locally aggressive with an extremely high rate of local recurrence. Either surgery or radiation alone often fails to cure the disease and the local failure is the main cause of treatment failure and death. Overall 5 year survival rate is less than 10%. Useful palliation or occasional cure can be obtained by the combination of surgery and radiotherapy. After incomplete resection, the tumor requires radiation dose of 7,000 cGy or more over 6-7 weeks for local control. Tumor regression is slow in response to irradiation and continuation of the regression for several months after completion of RT is not unusual. We report a case of chordoma of the thoracic vertebra, the site of extreme rarity, which showed good local control after partial resection and radiation therapy. He is well and alive without any evidence of recurrence after 13 months of treatment with near complete tumor regression.

  18. Radiation Therapy of a Chordoma of the Thoracic Vertebra-a Case Report and Review of Literatures-

    International Nuclear Information System (INIS)

    Kim, Joo Young; Choi, Myung Sun

    1988-01-01

    Chordom is a malignant tumor arising from the primitive notochord involving the axial skeleton. It usually occurs at sacrococcygeal and basisphenoidal area but only rarely does at other vertebral areas, especially at the thoracic vertebrae. It has a slow growth rate and is locally aggressive with an extremely high rate of local recurrence. Either surgery or radiation alone often fails to cure the disease and the local failure is the main cause of treatment failure and death. Overall 5 year survival rate is less than 10%. Useful palliation or occasional cure can be obtained by the combination of surgery and radiotherapy. After incomplete resection, the tumor requires radiation dose of 7,000 cGy or more over 6-7 weeks for local control. Tumor regression is slow in response to irradiation and continuation of the regression for several months after completion of RT is not unusual. We report a case of chordoma of the thoracic vertebra, the site of extreme rarity, which showed good local control after partial resection and radiation therapy. He is well and alive without any evidence of recurrence after 13 months of treatment with near complete tumor regression

  19. 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.

  20. Chordoma in the jugular foramen area: a review of 16 cases%颈静脉孔区脊索瘤16例临床分析

    Institute of Scientific and Technical Information of China (English)

    田凯兵; 王亮; 郝淑煜; 李达; 王科; 吴震; 张力伟; 张俊廷

    2013-01-01

    Objective To analysis the clinical characteristics,surgical treatment and outcome of chordoma in the jugular foramen area.Methods The author retrospectively studied data from 16 patients with chordoma in the jugular foramen area who had undergone surgeries in the department of skullbase and brain stem of Beijing Tiantan Hospital between January 2006 and June 2011,and follow-up was performed through recheck in out-patient department,telephone and letter.Results There are 8 females and 8 males in this group and all of them underwent surgical procedures.The mean age is 39-year-old and the mean symptomatic history time was 18.3 months.The main clinical presentation is paralysis of the lower cranial nerves (Ⅸ,Ⅹ and Ⅺ,12 cases),6 patients also suffered from deficits in cranial nerve Ⅷ,other presentations are deficits in eyesight (4 cases),ataxia (3 cases),numbness of limbs (2 cases),facial paralysis (2 cases) and deficits of abducent nerve (1 case).Far lateral approach was used in 10 cases,retrosigmoidsuboccipital approach was used in 2 cases,infratemporal trans-tentorium of cerebellum approach in 2 cases and expanded middle cranial fossa approach in 2 cases.Gross total removal was achivevd in 5 cases,subtotal removal in 10 cases and partial resection 1 case.The pathologic type of 5 cases in this group is typical chordoma,and other 11 cases are chondroid chordoma.Follow-up revealed obvious improvement in 5 cases,permanent paralysis of the lower cranial nerves in 7 cases and facial paralysis in 1 case.Conclusions The main clinical presentation of patients with jugular foramen chordoma is paralysis of the lower cranial nerves and the main pathology of the chordoma in this area is chondroid type,choosing an appropriate approach and the protection of lower cranial nerve are important for a good prognosis.%目的 探讨颈静脉孔区脊索瘤的临床特点和手术疗效.方法 回顾性分析16例颈静脉孔区脊索瘤患者的临

  1. 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

  2. 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

  3. 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

  4. 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

  5. [18F]-Fluoromisonidazole Positron Emission Tomography/Computed Tomography Visualization of Tumor Hypoxia in Patients With Chordoma of the Mobile and Sacrococcygeal Spine

    Energy Technology Data Exchange (ETDEWEB)

    Cheney, Matthew D., E-mail: mcheney@lroc.harvard.edu [Harvard Radiation Oncology Program, Boston, Massachusetts (United States); Chen, Yen-Lin [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Lim, Ruth [Department of Diagnostic Radiology, Massachusetts General Hospital, Boston, Massachusetts (United States); Winrich, Barbara K.; Grosu, Anca L.; Trofimov, Alexei V. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Depauw, Nicolas [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Centre of Medical Radiation Physics, University of Wollongong, Wollongong, NSW (Australia); Shih, Helen A. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Schwab, Joseph H.; Hornicek, Francis J. [Department of Orthopedic Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); DeLaney, Thomas F. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States)

    2014-12-01

    Purpose: To investigate [18F]-fluoromisonidazole positron emission tomography/computed tomography (FMISO-PET/CT) detection of targetable hypoxic subvolumes (HSVs) in chordoma of the mobile or sacrococcygeal spine. Methods and Materials: A prospective, pilot study of 20 patients with primary or locally recurrent chordoma of the mobile or sacrococcygeal spine treated with proton or combined proton/photon radiation therapy (RT) with or without surgery was completed. The FMISO-PET/CT was performed before RT and after 19.8-34.2 GyRBE (relative biologic effectiveness). Gross tumor volumes were delineated and HSVs defined including voxels with standardized uptake values ≥1.4 times the muscle mean. Clinical characteristics and treatments received were compared between patients with and without HSVs. Results: The FMISO-PET/CT detected HSVs in 12 of 20 patients (60%). Baseline and interval HSV spatial concordance varied (0%-94%). Eight HSVs were sufficiently large (≥5 cm{sup 3}) to potentially allow an intensity modulated proton therapy boost. Patients with HSVs had significantly larger gross tumor volumes (median 410.0 cm{sup 3} vs 63.4 cm{sup 3}; P=.02) and were significantly more likely to have stage T2 tumors (5 of 12 vs 0 of 8; P=.04). After a median follow-up of 1.8 years (range, 0.2-4.4 years), a local recurrence has yet to be observed. Three patients developed metastatic disease, 2 with HSVs. Conclusions: Detection of targetable HSVs by FMISO-PET/CT within patients undergoing RT with or without surgery for treatment of chordoma of the mobile and sacrococcygeal spine is feasible. The study's inability to attribute interval HSV changes to treatment, rapidly changing hypoxic physiology, or imaging inconsistencies is a limitation. Further study of double-baseline FMISO-PET/CT and hypoxia-directed RT dose escalation, particularly in patients at high risk for local recurrence, is warranted.

  6. Struniak w okolicy kości krzyżowej u 47 – letniej pacjentki – opis przypadku = Chordoma in the sacral spine in an 47-year-old woman – case report

    Directory of Open Access Journals (Sweden)

    Kamila Woźniak

    2016-09-01

    • 2.        Katedra i Klinika Rehabilitacji, Collegium Medicum im. Ludwika Rydygiera w Bydgoszczy, Uniwersytet Mikołaja Kopernika w Toruniu     Streszczenie   Struniaki (łac. chordoma są rzadkimi, histologicznie łagodnymi, lecz klinicznie złośliwymi nowotworami szkieletu osiowego. Mogą powstać w dowolnym miejscu w linii środkowej ciała, gdzie istniała struna grzbietowa (np. stok, nosogardziel, siodło i okolica okołosiodłowa, otwór wielki kręgi i okolica krzyżowo-ogonowa. Struniaki to wolno rosnące nowotwory, wykazujące miejscową agresywność w stosunku do kości i tkanek miękkich. Struniaka po raz pierwszy opisał w 1856 roku Virchow i Luschke. Z kolei w 1858 roku Muller stwierdził, że nowotwór może pochodzić z pozostałości embrionalnych prymitywnej struny grzbietowej tzw. chorda dorsalis. Autorzy przedstawiają opis przypadku klinicznego leczonego operacyjne w Klinice Neurochirurgii, Neurotraumatologii i Neurochirurgii Dziecięcej Szpitala Uniwersyteckiego nr 1 im. dr A. Jurasza w Bydgoszczy z rozpoznanym w badaniu histopatologicznym struniakiem.   Słowa kluczowe: struniak, chrodoma, nowotwór, szkielet osiowy, neurochirurgia.     Abstract   Chordomas are rare, histologically benign but clinically malignant tumors of the axial skeleton. They can occur anywhere in the midline of the body where there notochord (clivus, nasopharynx, saddle and surroundings parasellar, the foramen vertebrae and surroundings sacral area. Chordomas are slow-growing cancer, showing local aggressiveness relative to bone and soft tissue. Chordoma was first described in 1856 by Virchow and Luschke. In turn, in 1858 Muller found that the cancer can come from embryonic remnants of the primitive notochord called chorda dorsalis. The authors present a clinical case operating treated in the Department of Neurosurgery, Neurotraumatology and Pediatric Neurosurgery, Dr Antoni Jurasz University Hospital No.1 in Bydgoszcz histopathological diagnosed

  7. Skull base chordomas: treatment outcome and prognostic factors in adult patients following conformal treatment with 3D planning and high dose fractionated combined proton and photon radiation therapy

    Energy Technology Data Exchange (ETDEWEB)

    Munzenrider, J E; Hug, E; McManus, P; Adams, J; Efird, J; Liebsch, N J

    1995-07-01

    Purpose: To report treatment outcome and prognostic factors for local recurrence-free survival and overall survival in adult patients with skull base chordomas treated with 3D planning and high dose fractionated combined proton and photon radiation therapy. Methods and Materials: From 1975 through 1993, 132 adult patients with skull base chordomas were treated with fractionated combined proton and photon radiation therapy. Seventy five patients (57%) were male and 57 (43%) female. Age ranged from 19 to 80 years (median 45.5 years). All pathology was verified at MGH by a single pathologist. Ninety six had non-chondroid (NCC) and 36 chondroid chordomas (CC), respectively. Median prescribed dose was 68.7 CGE (CGE, Cobalt Gray-equivalent: proton Gy X RBE 1.1 + photon Gy), ranging from 36 to 79.2 CGE; 95% received {>=} 66.6 CGE. Between 70 and 100% of the dose was given with the 160 MeV proton beam at the Harvard Cyclotron. 3D CT-based treatment planning has been employed in all patients treated since 1980. Median follow-up was 46 months (range 2-158 months). Results: Treatment outcome was evaluated in terms of local recurrence-free survival (LRFS) and disease specific survival (DSS), as well as treatment-related morbidity. Local failure (LF), defined as progressive neurological deficit with definite increase in tumor volume on CT or MRI scan, occurred in 39 patients (29.5%). LF was more common among women than among men:(26(57)) (46%) vs (13(75)) (17%), respectively. Thirty three of the 39 LF were seen in non-chondroid chordoma patients, with 6 occurring in patients with the chondroid variant (34% of NCC and 17% of CC), respectively. Distant metastasis was documented in 8 patients. LRFS was 81 {+-} 5.8%, 59 {+-} 8.3%, and 43 {+-} 10.4%, and DSS was 94 {+-} 3.6%, 80 {+-} 6.7%, and 50 {+-} 10.7% at 36, 60, and 96 months, respectively, for the total group. LRFS and DSS were not significantly different for patients with NCC than those with CC (p > .05). Gender was

  8. 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.

  9. 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

  10. Spot-Scanning Proton Radiation Therapy for Pediatric Chordoma and Chondrosarcoma: Clinical Outcome of 26 Patients Treated at Paul Scherrer Institute

    Energy Technology Data Exchange (ETDEWEB)

    Rombi, Barbara [Center for Proton Therapy, Paul Scherrer Institute, Villigen (Switzerland); ATreP (Provincial Agency for Proton Therapy), Trento (Italy); Ares, Carmen, E-mail: carmen.ares@psi.ch [Center for Proton Therapy, Paul Scherrer Institute, Villigen (Switzerland); Hug, Eugen B. [Center for Proton Therapy, Paul Scherrer Institute, Villigen (Switzerland); ProCure Proton Therapy Center, Somerset, New Jersey (United States); Schneider, Ralf; Goitein, Gudrun; Staab, Adrian; Albertini, Francesca; Bolsi, Alessandra; Lomax, Antony J. [Center for Proton Therapy, Paul Scherrer Institute, Villigen (Switzerland); Timmermann, Beate [Center for Proton Therapy, Paul Scherrer Institute, Villigen (Switzerland); WestGerman Proton Therapy Center Essen (Germany)

    2013-07-01

    Purpose: To evaluate the clinical results of fractionated spot-scanning proton radiation therapy (PT) in 26 pediatric patients treated at Paul Scherrer Institute for chordoma (CH) or chondrosarcoma (CS) of the skull base or axial skeleton. Methods and Materials: Between June 2000 and June 2010, 19 CH and 7 CS patients with tumors originating from the skull base (17) and the axial skeleton (9) were treated with PT. Mean age at the time of PT was 13.2 years. The mean prescribed dose was 74 Gy (relative biological effectiveness [RBE]) for CH and 66 Gy (RBE) for CS, at a dose of 1.8-2.0 Gy (RBE) per fraction. Results: Mean follow-up was 46 months. Actuarial 5-year local control (LC) rates were 81% for CH and 80% for CS. Actuarial 5-year overall survival (OS) was 89% for CH and 75% for CS. Two CH patients had local failures: one is alive with evidence of disease, while the other patient succumbed to local recurrence in the surgical pathway. One CS patient died of local progression of the disease. No high-grade late toxicities were observed. Conclusions: Spot-scanning PT for pediatric CH and CS patients resulted in excellent clinical outcomes with acceptable rates of late toxicity. Longer follow-up time and larger cohort are needed to fully assess tumor control and late effects of treatment.

  11. 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

  12. 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

  13. Outcomes in Treatment for Intradural Spinal Cord Ependymomas

    International Nuclear Information System (INIS)

    Volpp, P. Brian; Han, Khanh; Kagan, A. Robert; Tome, Michael

    2007-01-01

    Purpose: Spinal cord ependymomas are rare tumors, accounting for <2% of all primary central nervous system tumors. This study assessed the treatment outcomes for patients diagnosed with spinal cord ependymomas within the Southern California Kaiser Permanente system. Methods and Materials: We studied 23 patients treated with surgery with or without external beam radiotherapy (EBRT). The local and distant control rates and overall survival rates were determined. Results: The overall local control, overall recurrence, and 9-year overall survival rate was 96%, 17.4%, and 63.9%, respectively. Conclusions: The results of our study indicate that en bloc gross total resection should be the initial treatment, with radiotherapy reserved primarily for postoperative cases with unfavorable characteristics such as residual tumor, anaplastic histologic features, or piecemeal resection. Excellent local control and overall survival rates can be achieved using modern microsurgical techniques, with or without local radiotherapy

  14. [An adult case of intradural lumbo-sacral lipoma].

    Science.gov (United States)

    Hatayama, T; Sakoda, K; Tokuda, Y; Uozumi, T

    1992-10-01

    A rare case of lumbo-sacral lipoma in an adult case is reported. A 55-year-old male was admitted to the Department of Neurosurgery, Mazda Hospital, after a history of one year of urinary incontinence. Neurologically, no motor or sensory disturbance of the lower extremities was found in this patient. MRI showed a mass with high signal intensity on T2-weighted image, located between L3 to S2 vertebral segments. Metrizamide-CT scan demonstrated the outline of this hypodense mass at the same location as shown on MRI image. A L3 through L5 laminectomy was performed and the tumor was subtotally removed. Microscopic examination revealed that the tumor mass was made up of mature lipoma cells. Postoperative course of the patient was uneventful. The urinary incontinence was improved slightly. No motor or sensory deficit was found. We thought that MRI was useful for the correct diagnosis of lumbosacral lipoma. And it is best managed by operative removal of the tumor as early as possible after it is diagnosed.

  15. 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 guide treatment decisions and design further clinical trials. Copyright © 2017 Elsevier Ltd. All rights reserved.

  16. Distinguishing benign notochordal cell tumors from vertebral chordoma

    International Nuclear Information System (INIS)

    Yamaguchi, Takehiko; Iwata, Jun; Sugihara, Shinsuke; McCarthy, Edward F.; Karita, Michiaki; Murakami, Hideki; Kawahara, Norio; Tsuchiya, Hiroyuki; Tomita, Katsuro

    2008-01-01

    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.)

  17. 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.

  18. 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.

  19. [Limitation of medical treatment and ethics in chronic recurrent Clivus chordoma].

    Science.gov (United States)

    Egger, Alexandra; Müller-Busch, H Christof

    2008-01-01

    In this paper ethical questions concerning the limitation and termination of medical treatment of comatose patients will be discussed on the basis of a case study. The team is confronted with extremely high communicative and ethical demands, since every person engaged in the treatment and care of the patient should take part in this decision making process. The final responsibility regarding the medical decisions, however, lies with the doctor in charge. In such cases advance directives or living wills are important and should be taken into consideration.

  20. 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.

  1. Extraskeletal myxoid chondrosarcoma presenting as an intradural spinal mass: report of a rare clinical presentation with an emphasis on differential diagnostic considerations

    Directory of Open Access Journals (Sweden)

    Priya Rao

    2014-12-01

    Full Text Available Extraskeletal myxoid chondrosarcoma is a rare soft tissue neoplasm that occurs predominantly in the soft tissues of the lower extremities. Herein we present a case of a 29 year old male who presented with bilateral femoral numbness believed to be the result of prior injury to his back. A magnetic resonance imaging revealed a mass in the T4-T5 epidural space compressing the spinal cord. Laminectomy was performed and the lesion removed piecemeal. The pathology specimen consisted of multiple fragments of dura involved by a myxoid neoplasm with a nodular growth pattern. The tumor cells were arranged in anastomosing cords and strands. Individual tumor cells were small, of uniform size and shape, with small hyperchromatic nuclei and scant eosinophilic cytoplasm. Immunohistochemical stains were performed which showed the tumor cells were diffusely positive for vimentin and focally positive for EMA, S-100 protein and cytokeratin, whereas they were negative for CD34 and CD99. Fluorescence in situ hybridization (FISH studies showed a clonal population of cells with re-arrangement of the EWSR1 locus, confirming the histologic impression of extraskeletal myxoid chondrosarcoma. This is the first report of a case of an extraskeletal myxoid chondrosarcoma arising from the dura, confirmed to have rearrangement of the EWSR1 gene by FISH. There have only been two other cases of dural based extraskeletal myxoid chondrosarcoma reported prior to our case. We also briefly review the published literature and discuss differential diagnostic considerations for this rare tumor.

  2. Intraspinal Pressure Monitoring in a Patient with Spinal Cord Injury Reveals Different Intradural Compartments: Injured Spinal Cord Pressure Evaluation (ISCoPE) Study.

    OpenAIRE

    Phang, I; Papadopoulos, MC

    2015-01-01

    BACKGROUND: We recently described a technique for monitoring intraspinal pressure (ISP) after traumatic spinal cord injury (TSCI). This is analogous to intracranial pressure monitoring after brain injury. We showed that, after severe TSCI, ISP at the injury site is elevated as the swollen cord is compressed against the dura. METHODS: In a patient with complete thoracic TSCI, we sequentially monitored subdural ISP above the injury, at the injury site, and below the injury intraoperatively. Pos...

  3. Temperatura, Oximetría Cerebral y Disfunción Cognitiva Postoperatoria (DCPO) en pacientes intervenidos de Prótesis Total de Rodilla (PTR) con anestesia intradural. Estudio prospectivo

    OpenAIRE

    Salazar Garcia, Fátima

    2015-01-01

    Introducción: La Disfunción cognitiva postoperatoria (DCPO) tiene una alta incidencia en pacientes de cirugía ortopédica. Durante la anestesia y la cirugía se produce un descenso progresivo de la temperatura. Esta hipotermia tiene efectos deletéreos pero también puede tener un efecto protector cerebral. Nosotros planteamos la hipótesis que la temperatura perioperatoria puede tener un impacto sobre la aparición de DCPO. Por otro lado, la monitorización de la saturación regional cerebral de...

  4. 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

  5. Chordome extra-axial : a propos d'une localisation latero tracheale ...

    African Journals Online (AJOL)

    Most chordomas arise in the sacrococcygeal and spheno-occipital region. Extranotochordal chordomas are extremely unusual. Case report : A 50 year women suffering from a left compressive cervical mass with a acute respiratory detress. in cervicotomy, it was an inextirpable tumor in contact with the left thyroid lobe and ...

  6. Neuroendoscopic Trans-Third Ventricular Approach for Surgical Management of Ecchordosis Physaliphora.

    Science.gov (United States)

    Adib, Sasan Darius; Bisdas, Sotirios; Bornemann, Antje; Schuhmann, Martin U

    2016-06-01

    We sought to report the successful surgical management of a case of ecchordosis physaliphora (EP) using a neuro-endoscopic trans-third ventricular approach (ETTVA) and to provide a current review of literature on EP. A 57-year-old man presenting with a 2-year history of diplopia due to right abducens nerve palsy and paresthesia of the left body underwent magnetic resonance imaging, which revealed a retroclival intracisternal lesion. The cystic lesion was considered to be most likely EP according to neuroradiologic features. The patient underwent an endoscopic trans-third ventricular resection. A pediatric endoscope was passed from a precoronal burr hole through the left lateral into the third ventricle. The floor of the third ventricle was opened by a 2-micron laser. This approach permitted us to expose the lesion in the retroclival cistern and follow up with a subtotal removal. Remnants of the capsule, which were firmly adherent to small pontine arteries and the left abducens nerve, were left. Histology confirmed EP. The patient recovered well from surgery, and symptoms regressed at clinical follow-up. The endoscopic approach for third ventriculostomy can also be used for the surgical management of retroclival lesions. However, a small pediatric endoscope with an angled view, which can be passed through the floor of the third ventricle without causing harm, is mandatory to explore all important structures in the narrow surgical space. Limitations in this delicate environment are firm adhesions to vessels and nerves because only 1-instrument manipulation is possible and bleeding must be avoided. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. 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.

  8. An arachnoid cyst presenting as an intramedullary tumour

    NARCIS (Netherlands)

    Willems, P. W.; van den Bergh, W. M.; Vandertop, W. P.

    2000-01-01

    A case of thoracic intradural extramedullary arachnoid cyst is presented in which an intramedullary low grade glioma was suspected preoperatively. The cyst was widely fenestrated and postoperatively, the patient experienced considerable improvement in her symptoms. As postoperative MRI studies also

  9. Prevention: Exercise

    Medline Plus

    Full Text Available ... and Intradural Tumors TREATMENTS Assessment Tools Lumbar and Cervical Discography Electrodiagnostic Testing MRI Radiographic Assessment for Back Pain Radiological Assessment of Spinal Disorders Repeated End-Range ...

  10. 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,...

  11. Stereotactic Body Radiotherapy for Head and Neck Tumors

    Science.gov (United States)

    2016-04-18

    Squamous Cell Carcinoma of the Head and Neck; Nasopharyngeal Carcinoma; Salivary Gland Cancer; Head and Neck Sarcoma; Paraganglioma of Head and Neck; Chordoma of Head and Neck; Chondrosarcoma of Head and Neck; Angiofibroma of Head and Neck

  12. CT and MRI of the skull base, including the cranial nerves

    International Nuclear Information System (INIS)

    Weber, A.L.

    1991-01-01

    Some considerations about nuclear magnetic resonance and computerized tomography, essential for examining skull base lesions are treated here, including the cranial nerves. Neoplasms such as meningiomas, adenomas, chordomas, chondrosarcomas and others tumors are also cited, mentioning some commentaries. (author)

  13. 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

  14. Benign notochordal lesions of the axial skeleton: a review and current appraisal

    International Nuclear Information System (INIS)

    Kyriakos, Michael

    2011-01-01

    At the 1996 meeting of the International Skeletal Society, an idea was put forth that there existed symptomatic lesions of the axial skeleton, morphologically different from chordoma, that were consistent with benign notochordal remnants (rests). A review of the embryological basis for this concept is made, along with an analysis of these lesions, termed giant notochordal rests or benign notochordal cell tumors, that have been reported in the intervening 15 years, with a commentary on their relationship, if any, to chordoma. (orig.)

  15. Perineural Spread of Renal Cell Carcinoma: A Case Illustration with a Proposed Anatomic Mechanism and a Review of the Literature.

    Science.gov (United States)

    Capek, Stepan; Krauss, William E; Amrami, Kimberly K; Parisi, Joseph E; Spinner, Robert J

    2016-05-01

    Perineural spread (PNS) is an unusual mechanism of tumor extension and has been typically reported in squamous cell carcinoma, adenocystic carcinoma, and desmoplastic melanoma. Our group has previously demonstrated PNS in rectal, prostate, bladder, and cervical cancer from the primary site along the autonomic nerves to the major somatic nerves and even intradurally. We believe similar principles apply to renal cell carcinoma (RCC) as well, despite the different anatomy. We performed a retrospective search to identify cases of intradural-extramedullary metastases of RCC caused by PNS. Strict anatomic and imaging inclusion criteria were defined: only lesions located between T6 and L3 were included, and PNS as a potential cause had to be supported by imaging evidence. Although 3 cases of spinal intradural metastases were identified, only one met our strict inclusion criteria. A 61-year-old woman developed a late intradural-extramedullary metastasis of RCC 16 years after the original diagnosis that we believe represents an example of visceral organ PNS. RCC can propagate via PNS from the primary tumor along the autonomic nerves to the aorticorenal, celiac, and mesenteric ganglia and then along the thoracic and lumbar splanchnic nerves to the corresponding spinal nerves and intradurally. We present radiologic evidence together with the review of the literature to support the premise that PNS of RCC not only occurs but goes unrecognized. Copyright © 2016 Elsevier Inc. All rights reserved.

  16. Chondrosarcoma of the temporal bone. Diagnosis and treatment of 13 cases and review of the literature

    International Nuclear Information System (INIS)

    Coltrera, M.D.; Googe, P.B.; Harrist, T.J.; Hyams, V.J.; Schiller, A.L.; Goodman, M.L.

    1986-01-01

    Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with multiple sclerosis, glomus jugulare tumors, meningioma, and chordomas. The cranial nerve palsies frequently observed with the tumors are related to the anatomic locations of the tumors. Thirteen patients with this entity are presented and the eleven other cases in the literature are reviewed. Histologically the tumors are low grade and exhibit myxoid features. The myxoid features must be differentiated from chordoma and chondroid chordoma. The tumor locations preclude surgical excision and conventional radiation therapy can cause unacceptable neurologic sequelae. Proton beam therapy has been effective in short-term results and appears capable of avoiding serious neurologic side effects

  17. 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.)

  18. Proton therapy for tumors of the skull base

    International Nuclear Information System (INIS)

    Munzenrider, J.E.; Liebsch, N.J.

    1999-01-01

    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.)

  19. Myxoid chondrosarcoma of sphenoid bone

    Directory of Open Access Journals (Sweden)

    Amit K Chowhan

    2012-01-01

    Full Text Available The myxoid variant of chondrosarcoma is usually seen in soft tissues where it is known as chordoid sarcoma or parachordoma. Rarely, it involves bone and when it does, cranial bones are the preferred location. This tumor is frequently amalgamated with the chondroid variant of chordoma, especially when the lesion occurs in the sphenoid bone/spheno-occipital region, because of their similar clinical presentations, anatomical locations, radiological findings, and mistaken histopathological features. It is essential to distinguish myxoid chondrosarcoma from the chondroid variant of chordoma, because of the different treatment protocol and prognostic importance. We present such a location-based diagnostic dilemma, solved successfully with ancillary immunohistochemistry.

  20. Ruptured cervical arteriovenous fistulas presenting with subarachnoid hemorrhage and quadriplegia: an uncommon case.

    Science.gov (United States)

    Liu, Chien-Liang; Su, Yung-Cheng; Chen, Chien-Chih; Chong, Chee-Fah; Wang, Tzong-Luen

    2008-02-01

    Nontraumatic subarachnoid hemorrhage is a neurologic emergency, and prompt treatment is necessary to avoid catastrophic result. We present a patient with subarachnoid hemorrhage caused by ruptured cervical intradural extramedullary arteriovenous fistulas, which rapidly progressed to quadriplegia. Because of the timely management, the patient had a good recovery. This is a rare but important case that emergency physicians should be aware of.

  1. 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, ...

  2. Magnetic resonance imaging in the management of suspected spinal canal disease in patients with known malignancy

    International Nuclear Information System (INIS)

    Loughrey, Gareth J.; Collins, Conor D.; Todd, Susan M.; Brown, Nicola M.; Johnson, Richard J.

    2000-01-01

    AIM: The aim of this study was to examine the spectrum of spinal canal disease in patients with known malignancy using magnetic resonance imaging (MRI). MATERIALS AND METHODS: One hundred and fifty-five patients underwent a total of 159 spinal MRI examinations over a three-year period. Patients were examined using a 1.0T magnet and a phased array surface spine coil. Sagittal T1 weighted spin echo and STIR sequences were routinely employed. Axial T1 and T2 weighted spin echo images were obtained at sites of identified pathology. Contrast enhanced sagittal and axial T1 weighted spin echo images were acquired when the unenhanced appearances did not correlate with the clinical findings or when the images suggested intradural or intramedullary disease. RESULTS: Malignant disease affecting the spinal cord or cauda equina was noted in 104/159 (65%) patients (extradural n= 78, intradural n= 20, intramedullary n= 7); one patient had evidence of both intradural and intramedullary deposits. Multiple levels of extradural cord/cauda equina compression were present in 18/78 patients (23%). The thoracic spine was the most frequently affected (74%). Bone elements were the major component of extradural compression in 11/78 patients (14%). Intradural metastases were multiple in 15/20 patients (75%). Four of the six solitary intramedullary metastases were situated in the conus medullaris. CONCLUSION: Magnetic resonance imaging of the entire spine is the investigation of choice in patients with known malignancy and suspected spinal canal disease. Contrast-enhanced images should be acquired when the unenhanced appearances do not correlate with the clinical findings or when they suggest intradural or intramedullary disease. Loughrey, G.J. (2000)

  3. Magnetic resonance imaging in the management of suspected spinal canal disease in patients with known malignancy

    Energy Technology Data Exchange (ETDEWEB)

    Loughrey, Gareth J.; Collins, Conor D.; Todd, Susan M.; Brown, Nicola M.; Johnson, Richard J

    2000-11-01

    AIM: The aim of this study was to examine the spectrum of spinal canal disease in patients with known malignancy using magnetic resonance imaging (MRI). MATERIALS AND METHODS: One hundred and fifty-five patients underwent a total of 159 spinal MRI examinations over a three-year period. Patients were examined using a 1.0T magnet and a phased array surface spine coil. Sagittal T1 weighted spin echo and STIR sequences were routinely employed. Axial T1 and T2 weighted spin echo images were obtained at sites of identified pathology. Contrast enhanced sagittal and axial T1 weighted spin echo images were acquired when the unenhanced appearances did not correlate with the clinical findings or when the images suggested intradural or intramedullary disease. RESULTS: Malignant disease affecting the spinal cord or cauda equina was noted in 104/159 (65%) patients (extradural n= 78, intradural n= 20, intramedullary n= 7); one patient had evidence of both intradural and intramedullary deposits. Multiple levels of extradural cord/cauda equina compression were present in 18/78 patients (23%). The thoracic spine was the most frequently affected (74%). Bone elements were the major component of extradural compression in 11/78 patients (14%). Intradural metastases were multiple in 15/20 patients (75%). Four of the six solitary intramedullary metastases were situated in the conus medullaris. CONCLUSION: Magnetic resonance imaging of the entire spine is the investigation of choice in patients with known malignancy and suspected spinal canal disease. Contrast-enhanced images should be acquired when the unenhanced appearances do not correlate with the clinical findings or when they suggest intradural or intramedullary disease. Loughrey, G.J. (2000)

  4. 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.

  5. Chondrosarcoma of the temporal bone: a case report

    International Nuclear Information System (INIS)

    Park, Man Soo; Lee, Sang Youl; Chung, Jae Gul; Lee, Deok Hee; Jung, Seung Mun; Ryu, Dae Sik

    2001-01-01

    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

  6. 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. 

  7. 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)

  8. Intramedullary capillary hemangioma of the thoracic spine: case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Paul Santiago

    2009-07-01

    Full Text Available Capillary hemangiomas are benign vascular neoplasms. When associated with the spine, these growths frequently involve the vertebral body, but rarely have they been reported to occur as intradural lesions, while even more rarely occurring in a true intramedullary location. We report a rare case of an intrame-dullary capillary hemangioma of the thoracic spinal cord and a review of the literature.

  9. 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.)

  10. Lumbosacral arachnoid cyst with tethered cord: A rare case report

    Directory of Open Access Journals (Sweden)

    S K Jain

    2012-01-01

    Full Text Available Arachnoid cysts are cerebrospinal fluid collections in the spine that can present with neurological symptoms or be discovered accidentally. Intradural location of such cysts especially in the lumbosacral region is relatively rare. The association of such cysts with other congenital anomalies such as tethered cord lends evidence to the developmental origin of arachnoid cysts. We report a case of lumbosacral arachnoid cyst with tethered cord in a 6-year-old male child and discuss the etiopathogenesis and management options.

  11. Microdialysis to optimize cord perfusion and drug delivery in spinal cord injury.

    OpenAIRE

    Phang, I; Zoumprouli, A; Papadopoulos, MC; Saadoun, S

    2016-01-01

    OBJECTIVE: There is lack of monitoring from the injury site to guide management of patients with acute traumatic spinal cord injury. Here we describe a bedside microdialysis monitoring technique for optimizing spinal cord perfusion and drug delivery at the injury site. METHODS: 14 patients were recruited within 72 hours of severe spinal cord injury. We inserted intradurally at the injury site a pressure probe, to monitor continuously spinal cord perfusion pressure, and a microdialysis cathete...

  12. Large Dumbbell-Shaped C1 Schwannoma Presenting as a Foramen Magnum Mass

    OpenAIRE

    Helms, Jody; Michael, Lattimore Madison

    2012-01-01

    Schwannomas involving the foramen magnum commonly originate from the lower cranial nerves, but they are rarely found arising from the first cervical root. To date, very few cases have been described in the literature. The majority involve either the intradural or extradural compartment but not both. We report the second case of a dumbbell-shaped schwannoma arising from the first cervical root. Our patient presented with hemisensory deficits secondary to brainstem compression at the level of t...

  13. 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)

  14. Giant thoracic schwannoma presenting with abrupt onset of abdominal pain: a case report

    Science.gov (United States)

    2009-01-01

    Introduction Giant intradural extramedullary schwannomas of the thoracic spine are not common. Schwannomas, that is, tumors derived from neoplastic Schwann cells, and neurofibromas represent the most common intradural extramedullary spinal lesions. We report the case of a patient with a giant thoracic schwannoma presenting unusually with acute abdominal pain and with delayed neurological impairment. Case presentation A 26-year-old Hispanic man with no previous medical problems presented with acute periumbilical pain. After extensive work-up including an exploratory laparotomy for appendectomy, magnetic resonance imaging scans of the lumbar and thoracic spine revealed a giant intradural extramedullary thoracic schwannoma within the spinal canal posterior to the T9, T10, and T11 vertebral bodies. Magnetic resonance imaging signal prolongation was noted in the spinal cord both rostral and caudal to the schwannoma. The patient underwent an urgent laminectomy from T8 to L1. After sacrificing the T10 root, the tumor was removed en bloc. Postoperatively, the patient improved significantly gaining antigravity strength in both lower extremities. Conclusion The T10 dermatome is represented by the umbilical region. This referred pain may represent a mechanism by which a giant thoracic schwannoma may present as acute abdominal pain. Acute, intense abdominal pain with delayed neurologic deficit is a rare presentation of a thoracic schwannoma but should be considered as a possible cause of abdominal pain presenting without clear etiology. Although these lesions may be delayed in their diagnosis, early diagnosis and treatment may lead to an improved clinical outcome. PMID:19946504

  15. Giant thoracic schwannoma presenting with abrupt onset of abdominal pain: a case report

    Directory of Open Access Journals (Sweden)

    Yang Isaac

    2009-10-01

    Full Text Available Abstract Introduction Giant intradural extramedullary schwannomas of the thoracic spine are not common. Schwannomas, that is, tumors derived from neoplastic Schwann cells, and neurofibromas represent the most common intradural extramedullary spinal lesions. We report the case of a patient with a giant thoracic schwannoma presenting unusually with acute abdominal pain and with delayed neurological impairment. Case presentation A 26-year-old Hispanic man with no previous medical problems presented with acute periumbilical pain. After extensive work-up including an exploratory laparotomy for appendectomy, magnetic resonance imaging scans of the lumbar and thoracic spine revealed a giant intradural extramedullary thoracic schwannoma within the spinal canal posterior to the T9, T10, and T11 vertebral bodies. Magnetic resonance imaging signal prolongation was noted in the spinal cord both rostral and caudal to the schwannoma. The patient underwent an urgent laminectomy from T8 to L1. After sacrificing the T10 root, the tumor was removed en bloc. Postoperatively, the patient improved significantly gaining antigravity strength in both lower extremities. Conclusion The T10 dermatome is represented by the umbilical region. This referred pain may represent a mechanism by which a giant thoracic schwannoma may present as acute abdominal pain. Acute, intense abdominal pain with delayed neurologic deficit is a rare presentation of a thoracic schwannoma but should be considered as a possible cause of abdominal pain presenting without clear etiology. Although these lesions may be delayed in their diagnosis, early diagnosis and treatment may lead to an improved clinical outcome.

  16. Metastatic disease of the spine: MR evaluation with Gd-DTPA

    International Nuclear Information System (INIS)

    Sze, G.; Abramson, A.; Krol, G.; Zimmerman, R.D.; Deck, M.D.F.

    1987-01-01

    Gd-DTPA was used in the MR evaluation of 22 patients with metastatic disease of the spine. Ten patients had epidural lesions; ten patients had intradural extramedullary spread of tumor; two patients had intramedullary metastases. A 1.5-T superconductive magnet with surface coils was used to obtain 3- or 5-mm sagittal and axial sections of the spine. All patients also received myelography and postmyelographic CT. Gd-DTPA was very sensitive to intradural extramedullary disease. Both nodular metastases and nerve root thickening enhanced. Lesions often poorly defined or not visible at all without contrast were easily seen after administration of gadolinium. Gadolinium was also of benefit in patients with intramedullary metastases. Although abnormalities of cord contour and signal were present in noncontrast studies, the region of enhancement was more localized and focal, suggesting that much of the cord enlargement was due to edema. In patients with extradural disease, gadolinium proved to be useful in selected cases. Generally, epidural tumor extension was visible without contrast. In specific cases, administration of gadolinium was beneficial, for example in localizing regions of tumor for biopsy or in distinguishing possible herniated nucleus pulposus from epidural disease. In conclusion, gadolinium is of significant benefit in intradural extramedullary disease. Metastatic lesions of the cord itself may also be more fully evaluated with contrast scans. However, epidural disease will probably prove to be adequately studied without contrast, although gadolinium may be of use in selected applications

  17. Experience with carbon ion radiotherapy at GSI

    Energy Technology Data Exchange (ETDEWEB)

    Jaekel, O. [Division of Medical Physics in Radiation Therapy (E040), German Cancer Research Center, Deutsches Krebsforschungszentrum, Im Neuenheimer Feld 280, 69120 Heidelberg (Germany)]. E-mail: o.jaekel@dkfz.de; Schulz-Ertner, D. [Department of Radiation Oncology, University of Heidelberg, Heidelberg (Germany); Karger, C.P. [Division of Medical Physics in Radiation Therapy (E040), German Cancer Research Center, Deutsches Krebsforschungszentrum, Im Neuenheimer Feld 280, 69120 Heidelberg (Germany); Heeg, P. [Division of Medical Physics in Radiation Therapy (E040), German Cancer Research Center, Deutsches Krebsforschungszentrum, Im Neuenheimer Feld 280, 69120 Heidelberg (Germany); Debus, J. [Department of Radiation Oncology, University of Heidelberg, Heidelberg (Germany)

    2005-12-15

    At GSI, a radiotherapy facility was established using beam scanning and active energy variation. Between December 1997 and April 2004, 220 patients have been treated at this facility with carbon ions. Most patients are treated for chordoma and chondrosarcoma of the base of skull, using a dose of 60 Gye (Gray equivalent) in 20 fractions. Carbon ion therapy is also offered in a combination with conventional radiotherapy for a number of other tumors (adenoidcystic carcinoma, chordoma of the cervical spine and sacrum, atypical menningeoma). The patients treated for skull base tumors showed an overall local control rate after two years of 90%. The overall treatment toxicity was mild. This shows that carbon ion radiotherapy can safely be applied using a scanned beam and encouraged the Heidelberg university hospital to build a hospital based facility for ion therapy.

  18. Diagnosis of pelvic wall tumor on multislice CT

    International Nuclear Information System (INIS)

    Zhang Keyun; Deng Lequn; Lei Hongwei

    2011-01-01

    Objective: To evaluate the value of multi-slice CT (MSCT) in diagnosing pelvic wall tumors. Methods: MSCT of 21 cases of pelvic wall tumors including metastasis (10), neurogenic tumor (5), chondrosarcoma (2), chordoma (1), aneurysmal bone cyst (1), giant cell tumor (1), and osteochondroma (1) was retrospectively analyzed. Results: CT appearances of pelvic wall tumors include bony destruction and soft tissue masses. Common features were bone destruction in metastasis, expansion of the neuroforamen in neurogenic tumor, pleomorphic calcification in chondrosarcoma, lower sacral vertebral location of chordoma, iliac crest bone destruction in giant cell tumor, cauliflower-like nodules in osteochondroma. Conclusion: MSCT with three-dimensional volume rendering demonstrates well the tumor shape, size, extent, internal structure and relationship with the surrounding organs to aid diagnosis of pelvic wall tumors. (authors)

  19. Acquired myospherulosis secondary to gluteal augmentation on fine needle aspiration cytology: A diagnostic challenge.

    Science.gov (United States)

    Alperstein, Susan; Dilcher, Thomas; Viswanathan, Kartik; Rao, Rema A; Siddiqui, Momin T; Giorgadze, Tamara

    2018-05-01

    A 30-year-old female presented with a three-month history of a multilocular cystic lesion over the lumbosacral spine. Fine-needle aspiration biopsy (FNA) of the lesion was performed at an outside institution, and a cytologic diagnosis, suspicious for chordoma, was rendered. The patient presented for surgical consultation at our institution. Repeat FNA demonstrated an unusual fat-like material. Upon further inquiry, the patient provided a recent history of gluteal contour improvement with fibroadipose tissue implants. A diagnosis of myospherulosis was made with a concurrent surgical pathology correlation. No evidence of chordoma was identified. To date, this is the first reported case of acquired myospherulosis in the context of gluteal contour enhancement and represents an important diagnostic pitfall to consider on cytology preparations. © 2017 Wiley Periodicals, Inc.

  20. 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

  1. 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...

  2. Sellar tumors; Tumoren der Sellaregion

    Energy Technology Data Exchange (ETDEWEB)

    Weber, M.A.; Essig, M. [Deutsches Krebsforschungszentrum, Heidelberg (Germany). Abt. Radiologie; Zoubaa, S. [GSF - Forschungszentrum fuer Gesundheit und Umwelt, Neuherberg, Muenchen (Germany). Abt. Neuropathologie; Politi, M.; Grunwald, I. [Universitaetsklinikum Saarland, Homburg (Germany). Klinik fuer Diagnostische und Interventionelle Neuroradiologie

    2007-06-15

    Because of the complex anatomy, examination of the sella turcica and hypophysis needs a dedicated MR sequence protocol. Not every sellar lesion is a pituitary adenoma. Thus, this review article summarizes the most frequently encountered intra-, supra-, and parasellar tumors and lesions. Differential diagnoses comprise besides adenoma among others craniopharyngioma, meningioma, glioma, germinoma, hamartoma, aneurysm, trigeminal schwannoma, pituitary carcinoma, chordoma, metastasis, infection, and empty sella. Characteristic imaging findings are presented and correlated to micro- and macro-pathology. (orig.)

  3. Notochord isolation using laser capture microdissection.

    Science.gov (United States)

    Santegoeds, R G C; Yakkioui, Y; Jahanshahi, A; Raven, G; Van Overbeeke, J J; Herrler, A; Temel, Y

    2017-03-01

    Chordoma are malignant tumors of the axial skeleton, which arise from remnants of the notochord. The Notochord (chorda dorsalis) is an essential embryonic structure involved in the development of the nervous system and axial skeleton. Therefore, the notochord seems to be the most biologically relevant control tissue to study chordoma in molecular biology research. Nevertheless, up to now mainly different tissues but not the notochord have been used as control for chordoma, due to difficulty of isolating notochordal tissue. Here, we describe a fast and precise method of isolating notochordal cells. Examination of human fetuses, with a gestation of 9, 11 and 13 weeks, using (immuno)histochemical methods was performed. To isolate pure notochord cells for further molecular biology investigation five flash frozen fetuses between 9 and 10 weeks of gestation were dissected by microtome slicing. Thereafter pure notochord cells for further molecular biology investigation where harvested by using laser capture microdissection (LCM). RNA was extracted from these samples and used in quantitative PCR. This study illustrates notochord of embryonic spines in three different stages of gestation (9-11-13 weeks). Immunohistochemical staining with brachyury showed strong staining of the notochord, but also weak staining of the intervertebral disc and vertebral body. LCM of notochord slices and subsequent total RNA extraction resulted in a good yield of total RNA. qPCR analysis of two housekeeping genes confirmed the quality of the RNA. LCM is a fast and precise method to isolate notochord and the quality and yield RNA extracted from this tissue is sufficient for qPCR analysis. Therefore early embryo notochord isolated by LCM is suggested to be the gold standard for future research in chordoma development, classification and diagnosis. Copyright © 2016 Elsevier B.V. All rights reserved.

  4. Radiological diagnostics of skeletal tumors

    International Nuclear Information System (INIS)

    Uhl, M.; Herget, G.W.

    2008-01-01

    The book contains contributions concerning the following topics: 1. introduction and fundamentals: WHO classification of bone tumors, imaging diagnostics and their function; localization, typical clinical and radiological criteria, TNM classification and status classification, invasive tumor diagnostics; 2. specific tumor diagnostics: chondrogenic bone tumors, osseous tumors, connective tissue bony tumors, osteoclastoma, osteomyelogenic bone tumors, vascular bone tumors, neurogenic bone tumors, chordoma; adamantinoma of the long tubular bone; tumor-like lesions, bony metastases, bone granulomas, differential diagnostics: tumor-like lesions

  5. 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

  6. Skull base chordoid meningioma: Imaging features and pathology

    International Nuclear Information System (INIS)

    Soo, Mark Y.S.; Gomes, Lavier; Ng, Thomas; Cruz, Malville Da; Dexter, Mark

    2004-01-01

    The clinical, imaging and pathological features of a skull base chordoid meningioma (CM) are described. The huge tumour resulted in obstructive hydrocephalus and partial erosion of the clivus such that a chordoma was suspected. The lesion's MRI findings were similar to those of a meningioma. Light microscopic, immunohistochemistry and ultrastructural features were diagnostic of CM. Chordoid meningioma is a rare subtype of meningioma and has a great tendency to recur should surgical resection be incomplete Copyright (2004) Blackwell Publishing Asia Pty Ltd

  7. [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

  8. Distribution and retention of 35S-sodium sulfate in man

    International Nuclear Information System (INIS)

    Woodard, H.Q.; Pentlow, K.S.; Mayer, K.; Laughlin, J.S.; Marcove, R.C.

    1976-01-01

    Measurements were made of the 35 S content of tissues obtained from biopsies and autopsies made during and up to 6 months after treatment of chondrosarcoma or chordoma with carrier-free Na 2 35 SO 4 . Usually 70 to 90 percent of an intravenous dose was excreted in the urine during the first 3 days. The major component of the blood concentration had a biologic half-time of 0.4 to 0.7 days. The initial uptakes in chondrosarcoma, chordoma, and red bone marrow were high and nearly equal, but the rates of loss differed greatly. Uptake in epiphyseal cartilage was comparable to that in chondrosarcoma; uptake in other types of cartilage was lower, but subsequent loss was very slow. For an administered dose of 1 mCi per kilogram of body weight, the integrated radiation doses were 2.4 rads for blood, 33 rads for red bone marrow, 155 rads for chondrosarcoma, 49 rads for chordoma, and 135 rads for normal cartilage. Doses to muscle, skin, and fibrous tissue were 7 to 15 rads

  9. Stereotactic gamma radiosurgery of brain tumors

    Energy Technology Data Exchange (ETDEWEB)

    Kobayashi, Tatsuya; Kida, Yoshihisa; Tanaka, Takayuki; Oyama, Hirofumi; Yoshida, Kazuo; Maesawa, Satoshi; Kai, Osamu; Nakamura, Mototoshi; Arahata, Masashige [Komaki City Hospital, Aichi (Japan)

    1996-06-01

    One thousand cases with various head and neck diseases have been treated by gamma radiosurgery at Komaki City Hospital since May 1991. Five hundred and sixty-eight out of 1,000 cases were neoplastic lesions which consisted of 173 cases of neurinoma, 108 of metastatic tumors, 103 of meningioma, 69 of gliomas, 27 of pituitary adenoma, 26 of craniopharyngioma, 13 of pineal tumors, 11 of chordoma, 6 of malignant lymphoma, 5 of hemangioblastoma and so on. The most effective result has been shown in metastatic brain tumors. The complete response (disappearance of the lesion) was obtained in more than 50% of the treated lesions, and the control rate of 85% was maintained for more than 12 months. Next effective results were shown in craniopharyngioma, malignant pineal tumors and malignant lymphoma. There was a group which showed moderate response but no tumor disappearance. Those were pituitary adenoma, acoustic neurinoma, meningioma and chordoma. Gliomas showed less response and even progression of tumor at relatively higher rate. It has been found that malignant gliomas showed difficult control of the tumor and progression rate of 70%, while benign gliomas showed the control rate of more than 90%. Besides intracranial lesions, malignant skull base tumors such as chordoma, naso-pharyngeal cancer, adenoid cystic cancer showed better response to gamma radiosurgery and higher control rate for longer period of time with high QOL compaired to conventional irradiation. (author)

  10. [Neurophysiological identification of the cranial nerves in endoscopic endonasal surgery of skull base tumors].

    Science.gov (United States)

    Shkarubo, A N; Ogurtsova, A A; Moshchev, D A; Lubnin, A Yu; Andreev, D N; Koval', K V; Chernov, I V

    2016-01-01

    Intraoperative identification of the cranial nerves is a useful technique in removal of skull base tumors through the endoscopic endonasal approach. Searching through the scientific literature found one pilot study on the use of triggered electromyography (t-EMG) for identification of the VIth nerve in endonasal endoscopic surgery of skull base tumors (D. San-Juan, et al, 2014). The study objective was to prevent iatrogenic injuries to the cranial nerves without reducing the completeness of tumor tissue resection. In 2014, 5 patients were operated on using the endoscopic endonasal approach. Surgeries were performed for large skull base chordomas (2 cases) and trigeminal nerve neurinomas located in the cavernous sinus (3). Intraoperatively, identification of the cranial nerves was performed by triggered electromyography using a bipolar electrode (except 1 case of chordoma where a monopolar electrode was used). Evaluation of the functional activity of the cranial nerves was carried out both preoperatively and postoperatively. Tumor resection was total in 4 out of 5 cases and subtotal (chordoma) in 1 case. Intraoperatively, the IIIrd (2 patients), Vth (2), and VIth (4) cranial nerves were identified. No deterioration in the function of the intraoperatively identified nerves was observed in the postoperative period. In one case, no responses from the VIth nerve on the right (in the cavernous sinus region) were intraoperatively obtained, and deep paresis (up to plegia) of the nerve-innervated muscles developed in the postoperative period. The nerve function was not impaired before surgery. The t-EMG technique is promising and requires further research.

  11. Effects of sex on the incidence and prognosis of spinal meningiomas: a Surveillance, Epidemiology, and End Results study.

    Science.gov (United States)

    Westwick, Harrison J; Shamji, Mohammed F

    2015-09-01

    Most spinal meningiomas are intradural lesions in the thoracic spine that present with both local pain and myelopathy. By using the large prospective Surveillance, Epidemiology, and End Results (SEER) database, the authors studied the incidence of spinal meningiomas and examined demographic and treatment factors predictive of death. Using SEER*Stat software, the authors queried the SEER database for cases of spinal meningioma between 2000 and 2010. From the results, tumor incidence and demographic statistics were computed; incidence was analyzed as a function of tumor location, pathology, age, sex, and malignancy code. Survival was analyzed by using a Cox proportional hazards ratio in SPSS for age, sex, marital status, primary site, size quartile, treatment modality, and malignancy code. In this analysis, significance was set at a p value of 0.05. The 1709 spinal meningiomas reported in the SEER database represented 30.7% of all primary intradural spinal tumors and 7.9% of all meningiomas. These meningiomas occurred at an age-adjusted incidence of 0.193 (95% CI 0.183-0.202) per 100,000 population and were closely related to sex (337 [19.7%] male patients and 1372 [80.3%] female patients). The Cox hazard function for mortality in males was higher (2.4 [95% CI1.7-3.5]) and statistically significant, despite the lower lesion incidence in males. All-cause survival was lowest in patients older than 80 years. Primary site and treatment modality were not significant predictors of mortality. Spinal meningiomas represent a significant fraction of all primary intradural spinal tumors and of all meningiomas. The results of this study establish the association of lesion incidence and survival with sex, with a less frequent incidence in but greater mortality among males.

  12. 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.).

  13. 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.

  14. 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.)

  15. 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.)

  16. 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.

  17. 12 Years delayed postoperative spinal recurrence of craniopharyngioma. Case report and literature review.

    Science.gov (United States)

    Marchesini, Nicolò; Soda, Christian; Ricci, Umberto Maria; Sedia, Mattia; Sala, Francesco; Pinna, Giampietro

    2017-12-04

    A case of delayed spinal adamantinomatous craniopharyngioma recurrence is presented. A 54-year-old male patient was admitted in our Emergency Department complaining of urinary disorders and leg pains. He underwent surgical removal of intraventricular craniopharyngioma 12 years previously. On MR imaging a well-circumscribed intradural cistyc mass at the T12 level was reavealed. A T11 and T12 laminotomy was performed and total removal of the tumour was achieved. Histology examination showed adamantinous craniopharyngioma. The authors believe that this represents the third case described of spinal craniofaryngioma recurrence and the first involving the dorsal spine.

  18. 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.

  19. 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

  20. Incidence and Prognosis of Spinal Hemangioblastoma: A Surveillance Epidemiology and End Results Study.

    Science.gov (United States)

    Westwick, Harrison J; Giguère, Jean-François; Shamji, Mohammed F

    2016-01-01

    Intradural spinal hemangioblastoma are infrequent, vascular, pathologically benign tumors occurring either sporadically or in association with von Hippel-Lindau disease along the neural axis. Described in fewer than 1,000 cases, literature is variable with respect to epidemiological factors associated with spinal hemangioblastoma and their treatment. The objective of this study was to evaluate the epidemiology of intradural spinal hemangioblastoma with the Surveillance, Epidemiology and End Results (SEER) database while also presenting an illustrative case. The SEER database was queried for cases of spinal hemangioblastoma between 2000 and 2010 with the use of SEER*Stat software. Incidence was evaluated as a function of age, sex and race. Survival was evaluated with the Cox proportionate hazards ratio using IBM SPSS software evaluating age, sex, location, treatment modality, pathology and number of primaries (p = 0.05). Descriptive statistics of the same factors were also calculated. The case of a 43-year-old patient with a surgical upper cervical intramedullary hemangioblastoma is also presented. In the data set between 2000 and 2010, there were 133 cases with an age-adjusted incidence of 0.014 (0.012-0.017) per 100,000 to the standard USA population. Hemangioblastoma was the tenth most common intradural spinal tumor type representing 2.1% (133 of 6,156) of all spinal tumors. There was no difference in incidence between men and women with an female:male rate ratio of 1.05 (0.73-1.50) with p = 0.86. The average age of patients was 48.0 (45.2-50.9) years, and a lower incidence was noted in patients incidence amongst the different races. Treatment included surgical resection in 106 (79.7%) cases, radiation with surgery in 7 (5.3%) cases, and radiation alone was used in only 1 (0.8%) case, and no treatment was performed in 17 (12.8%) cases. Mortality was noted in 12 (9%) cases, and median survival of 27.5 months (range 1-66 months) over the 10-year period. Mortality

  1. 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

  2. [The efficacy of desmopressin in the treatment of central diabetes insipidus after resection of chiasmo-sellar region tumors].

    Science.gov (United States)

    Astaf'eva, L I

    Central diabetes insipidus (CDI) is a neuroendocrine disease, the pathogenesis of which is associated with abnormal secretion of the antidiuretic hormone. One of the specific causes of CDI is neurosurgical resection of chiasmatic-sellar region tumors. to study the efficacy and safety of desmopressin in CDI patients after resection of chiasmatic-sellar region (CSR) tumors. Examination and treatment of patients were performed at a hospital for 7-14 days after surgery and then were continued after discharge. During treatment, the following tests were performed: a daily fluid intake and excretion volume, serum levels of sodium, potassium, and glucose twice a day, morning urine specific gravity, and Zimnitsky's test. Twenty-three patients with CSR tumors (11 craniopharyngiomas, 10 pituitary adenomas, 1 skull base chordoma, and 1 CSR meningioma) and CDI after neurosurgical treatment received desmopressin. On treatment, a thirst decrease, a reduced rate of diuresis, a reduced amount of excreted urine, and normalization of the sodium level were observed in all patients. In 12 patients (with pituitary adenoma, skull base chordoma, and meningioma) with transient CDI, desmopressin therapy was discontinued upon regression of symptoms 7-30 days after surgery. Eleven patients with permanent CDI continued to receive the drug at a dose of 1 to 4 doses per day. All patients well tolerated the drug without significant adverse effects. Therapy with desmopressin in the form of a nasal spray (vazomirin) in patients with transient and permanent CDI after resection CSR tumors of various histological nature (craniopharyngiomas, pituitary adenomas, meningiomas, and chordomas) was effective and safe in the early postoperative and long-term postoperative periods.

  3. Treatment with charged particles beams: hadron therapy part 1: physical basis and clinical experience of treatment with protons; Le traitement par faisceaux de particules: hadrontherapie 1: bases physiques et experience clinique de la protontherapie

    Energy Technology Data Exchange (ETDEWEB)

    Noel, G.; Feuvret, L.; Ferrand, R.; Mazeron, J.J. [Centre de Protontherapie d' Orsay, 91 (France); Mazeron, J.J. [Centre des Tumeurs, Groupe Hospitalier Universitaire Pitie-Salpetriere, 75 - Paris (France)

    2003-10-01

    Protons have physical characteristics, which differ from those of photons used in conventional radiotherapy. Better shielding of critical organs is obtained by using their particular ballistic (Bragg peak and lateral narrow penumbra). Some indications as ocular melanoma, chordoma and chondrosarcoma of the base of skull are now strongly accepted by the radiation oncologist community. Others are still in evaluation: meningioma, locally advanced nasopharynx tumor and paediatric tumors. The aim of this review is to present the clinical results of a technic which seems 'confidential' because of the rarity and the cost of equipments. (authors)

  4. 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

  5. Imaging findings of sacral tumors

    International Nuclear Information System (INIS)

    Kim, Seung Ho; Hong, Sung Hwan; Choi, Ja Young; Koh, Sung Hye; Chung, Hye Won; Choi, Jung Ah; Kang, Heung Sik

    2003-01-01

    The various pathologic conditions detected at CT and MRI and subsumed by the term 'sacral tumor' include primary bone tumors, sacral canal tumors and metastases. Among these, metastases are much more common than primary bone tumors, of which chordoma is the most common. Although the imaging findings of sacral tumors are nonspecific, a patient's age and sex, and specific findings such as calcification or fluid-fluid levels, can help radiologists in their differential diagnosis. We describe the imaging findings of primary sacral tumors, emphasizing the MRI findings

  6. Sacral Ewing's Sarcoma and Challenges in it's Diagnosis on MRI

    Directory of Open Access Journals (Sweden)

    Albert D'Souza

    2009-01-01

    Full Text Available A 15-yr old boy presented with low backache for 4 months associated with weakness of left lower limb. MRI of lumbosacral spine showed a sacral lesion with intraspinal and presacral soft tissue extension with neural compression. A diagnosis of tuberculosis was considered in the view of high prevalence in this part of the world, however biopsy revealed Ewing's sarcoma. Ewing's tumor of sacrum is rare, but should be suspected in low backache in children. Differential diagnosis for a sacral lesion includes tuberculosis, pyogenic osteomyelitis, lymphoma, chordoma, osteosarcoma and Ewing's sarcoma. MRI is sensitive in detecting these lesions but is nonspecific requiring histopathological examination for confirmation.

  7. 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.

  8. [Bath Plug Closure Method for Cerebrospinal Fluid Leakage by Endoscopic Endonasal Approach:Cooperative Treatment by Neurosurgeons and Otolaryngologists].

    Science.gov (United States)

    Kawaguchi, Tomohiro; Arakawa, Kazuya; Nomura, Kazuhiro; Ogawa, Yoshikazu; Katori, Yukio; Tominaga, Teiji

    2017-12-01

    Endoscopic endonasal surgery, an innovative surgical technique, is used to approach sinus lesions, lesions of the skull base, and intradural tumors. The cooperation of experienced otolaryngologists and neurosurgeons is important to achieve safe and reliable surgical results. The bath plug closure method is a treatment option for patients with cerebrospinal fluid(CSF)leakage. Although it includes dural and/or intradural procedures, surgery tends to be performed by otolaryngologists because its indications, detailed maneuvers, and pitfalls are not well recognized by neurosurgeons. We reviewed the cases of patients with CSF leakage treated by using the bath plug closure method with an endoscopic endonasal approach at our institution. Three patients were treated using the bath plug closure method. CSF leakage was caused by a meningocele in two cases and trauma in one case. No postoperative intracranial complications or recurrence of CSF leakage were observed. The bath plug closure method is an effective treatment strategy and allows neurosurgeons to gain in-depth knowledge of the treatment options for CSF leakage by using an endoscopic endonasal approach.

  9. 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

  10. 46-year-old man with a spinal cord mass.

    Science.gov (United States)

    Sanders, Mary Ann; Vitaz, Todd; Rosenblum, Marc; Plaga, Alexis R; Parker, Joseph C; Parker, John R

    2011-01-01

    Medulloblastoma accounts for only 1% of all adult CNS tumors. Likewise, recurrence of adult medulloblastoma greater than 20 years after initial diagnosis is extremely rare.We describe a case of adult medulloblastoma with late relapse of disease. The patient was 24 years old when first diagnosed and was treated with total tumor resection and craniospinal radiation. At the age of 45, an enhancing 1.3 cm intradural extramedullary spinal cord lesion at T5 was discovered on MRI. This was presumed to be recurrent medulloblastoma in the form of drop metastasis and the patient was treated with spinal radiation. Several months following treatment, at the age of 46, a follow-up MRI demonstrated an enhancing 1.4 cm intradural extramedullary spinal cord lesion at T7. The lesion was resected and histopathologic examination was most consistent with medulloblastoma, late drop metastasis. Although rare, adult medulloblastoma recurring 20 years after initial diagnosis should always be considered in the main differential diagnosis when working up CNS lesions at or outside the primary tumor site.

  11. 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.)

  12. 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.)

  13. Neurostimulation for bladder evacuation: is sacral root stimulation a substitute for microstimulation?

    Science.gov (United States)

    Probst, M; Piechota, H J; Hohenfellner, M; Gleason, C A; Tanagho, E A

    1997-04-01

    To determine by anatomical and functional studies whether stimulation of sacral rootlets might permit selective stimulation of autonomic fibres, thus avoiding the detrusor/sphincter dyssynergia characteristic of current techniques of neurostimulation for bladder evacuation. In 10 male mongrel dogs, the S2 root was isolated and its constituent rootlets followed from their origin in the spinal cord to the point of exit from the dura. The entire root and the individual rootlets were then stimulated, including intra- and extra-dural stimulation and at proximal, mid and distal levels. Neuroanatomical and histological findings showed that rootlets of ventral S2 maintain their identity throughout their intradural course; some carry predominantly autonomic fibres, some predominantly somatic and some a mixture of the two. It appears surgically feasible to identify, isolate and sever the predominantly somatic rootlets intradurally, sparing the predominantly autonomic rootlets for inclusion in extradural electrode placement around the entire sacral root, thus eliminating sphincteric interference with detrusor contraction for voiding at low pressure.

  14. Non-communicating extradural arachnoid cyst: a rare case report with review of literature

    Directory of Open Access Journals (Sweden)

    Gupta Asheesh Kumar

    2018-03-01

    Full Text Available Extradural arachnoid cysts in the spine are uncommon causes of spinal cord compression in the paediatric population that are thought to arise from congenital defects in the duramater. In most literatures it is describe that such cysts communicating with the intrathecal subarachnoid space through a small defect in the dura. In this case report we describe a case of a child who presented with spinal cord compression caused by a large spinal extradural arachnoid cyst that did not communicate with the intradural subarachnoid space. An 9 year-old girl presented with progressive lower-extremity weakness, myelopathy, and severe gait ataxia. Magnetic resonance imaging of the spine demonstrated a large extradural arachnoid cyst extending from T4 to T9. The patient underwent a thoracic laminectomy for en bloc resection of the spinal extradural arachnoid cyst. Intra-operatively, the dura was intact and there was no evidence of communication into the intradural subarachnoid space. Postoperatively, the patient’s motor strength and ambulation improved immediately, and no subsequent cerebrospinal fluid leak occurred.

  15. Concepts of nerve regeneration and repair applied to brachial plexus reconstruction.

    Science.gov (United States)

    Bertelli, Jayme Augusto; Ghizoni, Marcos Flávio

    2006-01-01

    Brachial plexus injury is a serious condition that usually affects young adults. Progress in brachial plexus repair is intimately related to peripheral nerve surgery, and depends on clinical and experimental studies. We review the rat brachial plexus as an experimental model, together with its behavioral evaluation. Techniques to repair nerves, such as neurolysis, nerve coaptation, nerve grafting, nerve transfer, fascicular transfer, direct muscle neurotization, and end-to-side neurorraphy, are discussed in light of the authors' experimental studies. Intradural repair of the brachial plexus by graft implants into the spinal cord and motor rootlet transfer offer new possibilities in brachial plexus reconstruction. The clinical experience of intradural repair is presented. Surgical planning in root rupture or avulsion is proposed. In total avulsion, the authors are in favor of the reconstruction of thoraco-brachial and abdomino-antebrachial grasping, and on the transfer of the brachialis muscle to the wrist extensors if it is reinnervated. Surgical treatment of painful conditions and new drugs are also discussed.

  16. Extreme lateral approach in a case of acute-onset quadriplegia due to high cervical neurenteric cyst.

    Science.gov (United States)

    Abhishek, Agrawal; Anushree, Agrawal; Patir, Rana; Sehgal, A D

    2007-01-01

    Spinal neurenteric cysts are very rare intradural developmental lesions, predominantly localized anterior to the cervical cord and arising from misplaced endodermal cells in the 3rd week of embryonic life. An acute onset of symptoms can occur due to hemorrhage, but has not as yet been reported in relation to infection in the cyst. We report an 18-year-old male who presented with a rapid onset of respiratory distress and quadriplegia over a period of 6 h requiring intubation and ventilatory support. There was no respiratory effort with a dense sensory level from the neck. MRI of the cervical spine revealed an intradural extramedullary ventrally located cystic lesion at the C(2-3) level. There was no evidence of systemic infection elsewhere in the body. The lesion was radically excised using an extreme lateral approach as it provided excellent visualization of the cyst, thus permitting safe dissection without retraction of the cord. The cyst was tense and the contents turbid, thick, creamy and mucinous without any evidence of fresh or altered blood. Histopathological examination confirmed the cyst to be a neurenteric cyst. The cystic fluid, which was sent for culture, grew coagulase-negative Staphylococcus aureus. This is the first documented case of rapid neurological deterioration due to infection in a neurenteric cyst. In spite of an early effective surgical management, outcome was poor due to the rapid and profound neurological deficit. Copyright (c) 2007 S. Karger AG, Basel.

  17. 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

  18. Innovative real CSF leak simulation model for rhinology training: human cadaveric design.

    Science.gov (United States)

    AlQahtani, Abdulaziz A; Albathi, Abeer A; Alhammad, Othman M; Alrabie, Abdulkarim S

    2018-04-01

    To study the feasibility of designing a human cadaveric simulation model of real CSF leak for rhinology training. The laboratory investigation took place at the surgical academic center of Prince Sultan Military Medical City between 2016 and 2017. Five heads of human cadaveric specimens were cannulated into the intradural space through two frontal bone holes. Fluorescein-dyed fluid was injected intracranialy, then endoscopic endonasal iatrogenic skull base defect was created with observation of fluid leak, followed by skull base reconstruction. The outcome measures included subjective assessment of integrity of the design, the ability of creating real CSF leak in multiple site of skull base and the possibility of watertight closure by various surgical techniques. The fluid filled the intradural space in all specimens without spontaneous leak from skull base or extra sinus areas. Successfully, we demonstrated fluid leak from all areas after iatrogenic defect in the cribriform plate, fovea ethmoidalis, planum sphenoidale sellar and clival regions. Watertight closure was achieved in all defects using different reconstruction techniques (overly, underlay and gasket seal closure). The design is simulating the real patient with CSF leak. It has potential in the learning process of acquiring and maintaining the surgical skills of skull base reconstruction before direct involvement of the patient. This model needs further evaluation and competence measurement as training tools in rhinology training.

  19. 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.)

  20. Rare case of primary spinal ependymomatosis occurring in a 26-year-old man: a case report

    Directory of Open Access Journals (Sweden)

    Kaliaperumal Chandrasekaran

    2009-10-01

    Full Text Available Abstract Introduction The authors report a rare case of primary spinal ependymomatosis in a young adult man. Multiple primary ependymomatous lesions were seen on magnetic resonance imaging and no anaplasia was identified on the surgical-pathological analysis. The aetio-pathological mechanism and surgical significance of this rare occurrence is discussed. Case presentation A 26-year-old man of Polish origin presented with a ten-day history of pain in the left leg and lower back. This was followed by difficulty in urinating and a decrease in sensation in both legs. Examination revealed pyramidal signs and mild weakness in both lower limbs. He had early sphincter involvement requiring catheterization. Magnetic resonance imaging of the brain was normal. However, that of the spinal cord revealed multiple intradural spinal lesions, both intra- and extramedullary, extending from the cervical cord down to the cauda equina roots. T12-L1 laminectomy was performed. Multiple intradural, extra- and intra-medullary tumors were seen. After the operation, the patient deteriorated with a sensory level at T4. Post-operative cranio-spinal radiotherapy was administered but there was no clinical improvement in the lower limbs. Conclusion Primary spinal ependymomatosis is a rare phenomenon involving multiple spinal segments in the absence of a primary intracranial tumor. Radical excision is unrealistic in this condition. Biopsy followed by radiotherapy is the preferred method of treatment.

  1. 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.

  2. Single-energy intensity modulated proton therapy

    Science.gov (United States)

    Farace, Paolo; Righetto, Roberto; Cianchetti, Marco

    2015-09-01

    In this note, an intensity modulated proton therapy (IMPT) technique, based on the use of high single-energy (SE-IMPT) pencil beams, is described. The method uses only the highest system energy (226 MeV) and only lateral penumbra to produce dose gradient, as in photon therapy. In the study, after a preliminary analysis of the width of proton pencil beam penumbras at different depths, SE-IMPT was compared with conventional IMPT in a phantom containing titanium inserts and in a patient, affected by a spinal chordoma with fixation rods. It was shown that SE-IMPT has the potential to produce a sharp dose gradient and that it is not affected by the uncertainties produced by metal implants crossed by the proton beams. Moreover, in the chordoma patient, target coverage and organ at risk sparing of the SE-IMPT plan resulted comparable to that of the less reliable conventional IMPT technique. Robustness analysis confirmed that SE-IMPT was not affected by range errors, which can drastically affect the IMPT plan. When accepting a low-dose spread as in modern photon techniques, SE-IMPT could be an option for the treatment of lesions (e.g. cervical bone tumours) where steep dose gradient could improve curability, and where range uncertainty, due for example to the presence of metal implants, hampers conventional IMPT.

  3. Single-energy intensity modulated proton therapy.

    Science.gov (United States)

    Farace, Paolo; Righetto, Roberto; Cianchetti, Marco

    2015-10-07

    In this note, an intensity modulated proton therapy (IMPT) technique, based on the use of high single-energy (SE-IMPT) pencil beams, is described.The method uses only the highest system energy (226 MeV) and only lateral penumbra to produce dose gradient, as in photon therapy. In the study, after a preliminary analysis of the width of proton pencil beam penumbras at different depths, SE-IMPT was compared with conventional IMPT in a phantom containing titanium inserts and in a patient, affected by a spinal chordoma with fixation rods.It was shown that SE-IMPT has the potential to produce a sharp dose gradient and that it is not affected by the uncertainties produced by metal implants crossed by the proton beams. Moreover, in the chordoma patient, target coverage and organ at risk sparing of the SE-IMPT plan resulted comparable to that of the less reliable conventional IMPT technique. Robustness analysis confirmed that SE-IMPT was not affected by range errors, which can drastically affect the IMPT plan.When accepting a low-dose spread as in modern photon techniques, SE-IMPT could be an option for the treatment of lesions (e.g. cervical bone tumours) where steep dose gradient could improve curability, and where range uncertainty, due for example to the presence of metal implants, hampers conventional IMPT.

  4. Single-energy intensity modulated proton therapy

    International Nuclear Information System (INIS)

    Farace, Paolo; Righetto, Roberto; Cianchetti, Marco

    2015-01-01

    In this note, an intensity modulated proton therapy (IMPT) technique, based on the use of high single-energy (SE-IMPT) pencil beams, is described.The method uses only the highest system energy (226 MeV) and only lateral penumbra to produce dose gradient, as in photon therapy. In the study, after a preliminary analysis of the width of proton pencil beam penumbras at different depths, SE-IMPT was compared with conventional IMPT in a phantom containing titanium inserts and in a patient, affected by a spinal chordoma with fixation rods.It was shown that SE-IMPT has the potential to produce a sharp dose gradient and that it is not affected by the uncertainties produced by metal implants crossed by the proton beams. Moreover, in the chordoma patient, target coverage and organ at risk sparing of the SE-IMPT plan resulted comparable to that of the less reliable conventional IMPT technique. Robustness analysis confirmed that SE-IMPT was not affected by range errors, which can drastically affect the IMPT plan.When accepting a low-dose spread as in modern photon techniques, SE-IMPT could be an option for the treatment of lesions (e.g. cervical bone tumours) where steep dose gradient could improve curability, and where range uncertainty, due for example to the presence of metal implants, hampers conventional IMPT. (note)

  5. 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.)

  6. Putative oncogene Brachyury (T) is essential to specify cell fate but dispensable for notochord progenitor proliferation and EMT.

    Science.gov (United States)

    Zhu, Jianjian; Kwan, Kin Ming; Mackem, Susan

    2016-04-05

    The transcription factor Brachyury (T) gene is expressed throughout primary mesoderm (primitive streak and notochord) during early embryonic development and has been strongly implicated in the genesis of chordoma, a sarcoma of notochord cell origin. Additionally, T expression has been found in and proposed to play a role in promoting epithelial-mesenchymal transition (EMT) in various other types of human tumors. However, the role of T in normal mammalian notochord development and function is still not well-understood. We have generated an inducible knockdown model to efficiently and selectively deplete T from notochord in mouse embryos. In combination with genetic lineage tracing, we show that T function is essential for maintaining notochord cell fate and function. Progenitors adopt predominantly a neural fate in the absence of T, consistent with an origin from a common chordoneural progenitor. However, T function is dispensable for progenitor cell survival, proliferation, and EMT, which has implications for the therapeutic targeting of T in chordoma and other cancers.

  7. Tuberculum sellae meningiomas: surgical considerations Meningiomas do tuberculo selar: aspectos cirúrgicos

    Directory of Open Access Journals (Sweden)

    José Alberto Landeiro

    2010-06-01

    Full Text Available We report our experience on the treatment of tuberculum sellae meningiomas (TSMs regarding the involvement of the optic canal and clinical outcomes. We reviewed 23 patients who were operated on between January 1997 and December 2008. The surgical approach was unilateral subfrontal supraorbital osteotomy in one piece. Attempts were made to improve visual function via extra/intradural unroofing of the optic canal, which released the optic nerve. Visual symptoms were present preoperatively in 21 patients, and two patients were asymptomatic. Visual acuity remained intact in 6 patients, improved in 10, was unchanged in 5 patients, and worsened in 2 patients. The postoperative visual field was normal or improved in 17 patients, unchanged in four patients, and worsened in two patients. The optic canal and clinoid were drilled extradurally in eight patients and intradurally in nine patients. Total resection of TSMs was achieved in 19 patients. Incomplete resection occurred in two patients. Decompression of the optic canal seemed to increase the visual outcome.Apresentamos nossa experiência em 23 pacientes operados com meningiomas do tubérculo da sela, com enfoque na descompressão do nervo óptico e nos sintomas visuais do pós-operatório. Vinte e três pacientes com meningiomas do tubérculo da sela foram operados entre janeiro de 1997 e dezembro de 2008, através do acesso subfrontal via ostetomia supraorbital. Remoção do teto do canal óptico por via extra ou intradural foi realizada em 17 pacientes. Sintomas visuais no pré-operatório ocorreram em 21 pacientes, 2 eram assintomáticos. Melhora visual ocorreu em 10, permaneceu inalterada em 5 e piorou em 2 pacientes. No pós-operatório o campo visual normalizou-se em 17 pacientes, permaneceu inalterado em 4 e diminuiu em 2. Descompressão dos nervos ópticos foi realizada em 17 pacientes. Ressecção total dos meningiomas do tubérculo da sela foi possível em 19 pacientes. Abertura do canal

  8. 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

  9. 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

  10. Spot Scanning Proton Therapy for Malignancies of the Base of Skull: Treatment Planning, Acute Toxicities, and Preliminary Clinical Outcomes

    Energy Technology Data Exchange (ETDEWEB)

    Grosshans, David R., E-mail: dgrossha@mdanderson.org [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Zhu, X. Ronald; Melancon, Adam [Department of Radiation Physics, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Allen, Pamela K. [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Poenisch, Falk; Palmer, Matthew [Department of Radiation Physics, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); McAleer, Mary Frances; McGovern, Susan L. [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Gillin, Michael [Department of Radiation Physics, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); DeMonte, Franco [Department of Neurosurgery, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Chang, Eric L. [Department of Radiation Oncology, University of Southern California Keck School of Medicine, Los Angeles, California (United States); Brown, Paul D.; Mahajan, Anita [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States)

    2014-11-01

    Purpose: To describe treatment planning techniques and early clinical outcomes in patients treated with spot scanning proton therapy for chordoma or chondrosarcoma of the skull base. Methods and Materials: From June 2010 through August 2011, 15 patients were treated with spot scanning proton therapy for chordoma (n=10) or chondrosarcoma (n=5) at a single institution. Toxicity was prospectively evaluated and scored weekly and at all follow-up visits according to Common Terminology Criteria for Adverse Events, version 3.0. Treatment planning techniques and dosimetric data were recorded and compared with those of passive scattering plans created with clinically applicable dose constraints. Results: Ten patients were treated with single-field-optimized scanning beam plans and 5 with multifield-optimized intensity modulated proton therapy. All but 2 patients received a simultaneous integrated boost as well. The mean prescribed radiation doses were 69.8 Gy (relative biological effectiveness [RBE]; range, 68-70 Gy [RBE]) for chordoma and 68.4 Gy (RBE) (range, 66-70) for chondrosarcoma. In comparison with passive scattering plans, spot scanning plans demonstrated improved high-dose conformality and sparing of temporal lobes and brainstem. Clinically, the most common acute toxicities included fatigue (grade 2 for 2 patients, grade 1 for 8 patients) and nausea (grade 2 for 2 patients, grade 1 for 6 patients). No toxicities of grades 3 to 5 were recorded. At a median follow-up time of 27 months (range, 13-42 months), 1 patient had experienced local recurrence and a second developed distant metastatic disease. Two patients had magnetic resonance imaging-documented temporal lobe changes, and a third patient developed facial numbness. No other subacute or late effects were recorded. Conclusions: In comparison to passive scattering, treatment plans for spot scanning proton therapy displayed improved high-dose conformality. Clinically, the treatment was well tolerated, and

  11. Combined spinal intramedullary arteriovenous malformation and lipomyelomeningocele

    Energy Technology Data Exchange (ETDEWEB)

    Weon, Y.C.; Roh, H.G.; Byun, H.S. [Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Radiology, Seoul (Korea); Chung, J.I. [Medimoa Hospital, Department of Radiology, Seoul (Korea); Eoh, W. [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea)

    2005-10-01

    Combined spinal arteriovenous malformation and lipomyelomeningocele are extremely rare. We present a rare combined case of a lipomyelomeningocele with an intramedullary arteriovenous malformation (AVM) occurred at the L3-L4 level in a 30-year-old man who suffered from low back pain radiating to the lower extremities, dysuria, and frequency for 5 years. The MR studies showed an intradural mass with high-signal intensity on both T1-weighted and T2-weighted images, intermingled with multiple signal-void structures. The mass extended extradurally toward a subcutaneously forming fatty mass on the patient's back. Spinal angiography showed an AVM supplied by the radiculopial branches of the lumbar arteries and drained by tortuous, dilated, perimedullary veins. Endovascular embolization and surgical resection were performed. (orig.)

  12. 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...

  13. 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

  14. Fast-gradient-echo variable-flip-angle imaging of the cervical spine

    International Nuclear Information System (INIS)

    Van Dyke, C.W.; Ross, J.S.; Masaryk, T.J.; Tkach, J.; Beale, S.; Hueftle, M.G.; Kaufman, B.; Modic, M.T.

    1987-01-01

    Two hundred consecutive patients were studied with 4-mm sagittal and axial T1-weighted images and gradient echo sequences with 6-msec or 13-msec echo time (TE) and 10 0 or 60 0 flip angles to evaluate cervical extradural disease. Images were independently evaluated for contrast behavior and anatomy, then directly compared for conspicuity of lesions. FLASH sequences produced better conspicuity of disease in half the imaging time. T1-weighted spin-echo (SE) sequences were more sensitive to marrow changes and intradural disease. Shorter TEs produced overall image improvement and reduced susceptibility effects. A fast and sensitive cervical examination combines sagittal T1-weighted SE with sagittal and axial FLASH 10 0 sequences with 6-msec TE

  15. 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.)

  16. 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.

  17. 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)

  18. 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.

  19. 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.

  20. [Cervical myelopathy from an aspect of a neurological surgeon].

    Science.gov (United States)

    Koyama, T

    1994-12-01

    More than 65 per cent of intramedurally tumors and intradural extramedurally tumors were located in cervical region. They enact a cause of cervical myelopathy. A special attention should be paid to solitaly intramedurally vascular malformations, when the patients show an acute progressive myelopathy. In very rare cases of syringomyelia, intramedurally tumors such as hemangioblastoma or ependymoma could be found, so that enhanced MRI or angiography should be done if necessary. In cases of a herniated disc, cervical spondylosis and OPLL, the distance between articulo-pillar line and spinolaminal line must be measured. If the distance of both lines is near or the same, that means narrow cervical canal. In rare cases the form of the vertebral body is round. This is a rare cause of a type of narrow canal.

  1. Perianal pain as a presentation of lumbosacral neurofibroma: a case report.

    Science.gov (United States)

    Moghaddasi, Mehdi; Aghaii, Mahboubeh; Mamarabadi, Mansoureh

    2014-12-01

    Rectal and perianal pain is a common problem. Most people have experienced it at least once in their lifetime. It usually manifests as mild discomfort, but sometimes the pain can be so severe that it is incapacitating. A 59-year-old woman admitted with a 2-year history of paroxysmal perianal pain underwent a full work-up including proctoscopy, sigmoidoscopy, full colonoscopy, and barium enema that were unremarkable. Lumbosacral magnetic resonance imaging with and without gadolinium showed an intradural-extramedullary lesion at the level of L5. The pathologic diagnosis was a neurofibroma. She underwent surgery, and after a few weeks she felt well and medication was no longer needed for her paroxysmal pain. Although one should consider the usual causes of colorectal pain such as hemorrhoids, anal fissure, proctalgia fugax, and chronic perianal pain syndrome, we should keep in mind that some referral pain may mimic local pathologies and should be evaluated properly.

  2. Femoral nerve compression syndrome with paresis of the quadriceps muscle caused by radiotherapy of malignant tumours. A report of four cases

    Energy Technology Data Exchange (ETDEWEB)

    Laurent, L E [Orthopaedic Hospital of the Invalid Foundation, Helsinki, Finland

    1975-01-01

    Four patients showed signs of femoral nerve compression with subsequent paresis of the quadriceps muscle, after radiation therapy of malignant tumours. The compression was caused by scar tissue due to radiation treatment of the inguinal region. The first symptom was radiating pain in the front of the thigh and lower leg which appeared 12-16 months after X-ray treatment. A decrease in the strength of quadriceps muscle occurred some months later. In one case the femoral nerve was decompressed, another patient was treated by an intradural phenolglycerin injection and one patient was treated with cortisone and oxiphenbutanzone. In these cases the pain decreased considerably, but in one case only the paresis of the quadriceps muscle improved after treatment.

  3. 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.

  4. Large Dumbbell-Shaped C1 Schwannoma Presenting as a Foramen Magnum Mass

    Science.gov (United States)

    Helms, Jody; Michael, Lattimore Madison

    2012-01-01

    Schwannomas involving the foramen magnum commonly originate from the lower cranial nerves, but they are rarely found arising from the first cervical root. To date, very few cases have been described in the literature. The majority involve either the intradural or extradural compartment but not both. We report the second case of a dumbbell-shaped schwannoma arising from the first cervical root. Our patient presented with hemisensory deficits secondary to brainstem compression at the level of the foramen magnum. The patient underwent a far lateral approach, and a gross total resection was achieved. Preoperative suspicion of the diagnosis is helpful in anticipating displacement and avoiding damage to the surrounding neurovascular structures. PMID:23946923

  5. Tension pneumocephalus: Mount Fuji sign

    Directory of Open Access Journals (Sweden)

    Pulastya Sanyal

    2015-01-01

    Full Text Available A 13-year-old male was operated for a space occupying lesion in the brain. A noncontrast computed tomography scan done in the late postoperative period showed massive subdural air collection causing compression of bilateral frontal lobes with widening of interhemispheric fissure and the frontal lobes acquiring a peak like configuration - causing tension pneumocephalus-"Mount Fuji sign." Tension pneumocephalus occurs when air enters the extradural or intradural spaces in sufficient volume to exert a mass or pressure effect on the brain, leading to brain herniation. Tension pneumocephalus is a surgical emergency, which needs immediate intervention in the form of decompression of the cranial cavity by a burr hole or needle aspiration. The Mount Fuji sign differentiates tension pneumocephalus from pneumocephalus.

  6. 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.

  7. Subarachnoid Hemorrhage due to Spinal Cord Schwannoma Presenting Findings Mimicking Meningitis.

    Science.gov (United States)

    Zhang, Hong-Mei; Zhang, Yin-Xi; Zhang, Qing; Song, Shui-Jiang; Liu, Zhi-Rong

    2016-08-01

    Subarachnoid hemorrhage (SAH) of spinal origin is uncommon in clinical practice, and spinal schwannomas associated with SAH are even more rarely reported. We report an unusual case of spinal SAH mimicking meningitis with normal brain computed tomography (CT)/magnetic resonance imaging (MRI) and negative CT angiography. Cerebrospinal fluid examination results were consistent with the manifestation of SAH. Spinal MRI performed subsequently showed an intradural extramedullary mass. The patient received surgery and was finally diagnosed with spinal cord schwannoma. A retrospective chart review of the patient was performed. We describe a case of SAH due to spinal cord schwannoma. Our case highlights the importance of careful history taking and complete evaluation. We emphasize that spinal causes should always be ruled out in patients with angionegative SAH and that schwannoma should be considered in the differential diagnosis of SAH etiologies even though rare. Copyright © 2016 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  8. 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.

  9. 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.

  10. Meningioma of the scapula

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    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.)

  11. Multifocal Spinal Cord Nephroblastoma in a Dog.

    Science.gov (United States)

    Henker, L C; Bianchi, R M; Vargas, T P; de Oliveira, E C; Driemeier, D; Pavarini, S P

    2018-01-01

    A 1-year-old male American pit bull terrier was presented with a history of proprioceptive deficits and mild lameness of the right hindlimb, which progressed after 5 months to paraparesis, culminating in tetraparesis after 2 weeks. Necropsy findings were limited to the spinal cord and consisted of multiple, intradural, extramedullary, slightly red masses which produced segmental areas of medullary swelling located in the cervical intumescence, thoracolumbar column, sacral segment and cauda equina. Histological evaluation revealed a tumour, composed of epithelial, stromal and blastemal cells, with structures resembling tubules, acini and embryonic glomeruli. Immunohistochemical labelling for vimentin, cytokeratin and S100 was positive for the stromal, epithelial and blastemal cells, respectively. A final diagnosis of multifocal spinal cord nephroblastoma was established. This is the first report of such a tumour showing concomitant involvement of the cervicothoracic, thoracolumbar, sacral and cauda equina areas of the spinal cord. Copyright © 2017 Elsevier Ltd. All rights reserved.

  12. Astrocitoma frontal com metastase medular: registro de um caso e revisão da literatura

    Directory of Open Access Journals (Sweden)

    J. Jorge Facure

    1978-12-01

    Full Text Available Relato de um caso de astrocitoma grau II com metástase medular ao nível da terceira vértebra dorsal. O nódulo intradural determinou sinais e sintomas de compressão medular. O diagnóstico clínico foi confirmado por mielografia descendente com lipiodol e mielocintilografia com I131 - albumina. A paciente foi submetida a laminectomia para exerese da metástase. A revisão da literatura mostra que o fator mais importante para a ocorrência das metástase dos tumores encefálicos é a invasão do sistema ventricular e/ou espaço subaracnóideo periencefálico pela neoplasia, sem relação com o ato operatório.

  13. 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.

  14. 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

  15. Management of postoperative pseudomeningoceles: an international survey study.

    Science.gov (United States)

    Tu, Albert; Tamburrini, Gianpiero; Steinbok, Paul

    2014-11-01

    Pseudomeningoceles are common complications after posterior fossa and intradural spinal surgery and are often asymptomatic. Management guidelines are lacking, and anecdotally, we have encountered radically different suggested treatments varying from observation to immediate surgical intervention. The goal of this study was to determine the prevailing opinions among neurosurgeons on the management of this condition. Neurosurgeons from around the world were invited via an International Society for Pediatric Neurosurgery (ISPN) and Neurosurgery ListServ e-blast to participate in a 33-question survey on the management of pseudomeningoceles, presented as simulated scenarios after posterior fossa and spinal intradural surgery. Two hundred forty-one responses were obtained. Pseudomeningoceles after posterior fossa tumor resection, in the absence of hydrocephalus, were typically managed nonoperatively for 7 to 14 days before re-exploration. Only 0.5 % of the surgeons would offer upfront repair of the pseudomeningocele. In the presence of hydrocephalus, 48 % of the neurosurgeons intervene initially with cerebrospinal fluid (CSF) diversion and would change therapy if the lesion did not resolve in 2 to 4 days. Ninety percent of the surgeons manage spinal pseudomeningoceles nonoperatively for 7-14 days before re-exploration is considered. The most common steps taken to prevent pseudomeningoceles are watertight closure, tissue glues, and duroplasty. The present zeitgeist suggests that, in the absence of hydrocephalus, initial observation is appropriate for cranial and spinal pseudomeningoceles. Operative revision should be reserved for failure of conservative treatment. If hydrocephalus is present, consideration should be made for CSF diversion. This study may serve as a guideline regarding acceptable management.

  16. 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

  17. Lower cranial nerves function after surgical treatment of Fisch Class C and D tympanojugular paragangliomas.

    Science.gov (United States)

    Bacciu, Andrea; Medina, Marimar; Ait Mimoune, Hassen; D'Orazio, Flavia; Pasanisi, Enrico; Peretti, Giorgio; Sanna, Mario

    2015-02-01

    The aim of this study was to report the postoperative lower cranial nerves (LCNs) function in patients undergoing surgery for tympanojugular paraganglioma (TJP) and to evaluate risk factors for postoperative LCN dysfunction. A retrospective case review of 122 patients having Fisch class C or D TJP, surgically treated from 1988 to 2012, was performed. The follow-up of the series ranged from 12 to 156 months (mean, 39.4 ± 32.6 months). The infratemporal type A approach was the most common surgical procedure. Gross total tumor removal was achieved in 86% of cases. Seventy-two percent of the 54 patients with preoperative LCN deficit had intracranial tumor extension. Intraoperatively, LCNs had to be sacrificed in 63 cases (51.6%) due to tumor infiltration. Sixty-six patients (54.09%) developed a new deficit of one or more of the LCNs. Of those patients who developed new LCN deficits, 23 of them had intradural extension. Postoperative follow-up of at least 1 year showed that the LCN most commonly affected was the CN IX (50%). Logistic regression analysis showed that intracranial transdural tumor extension was correlated with the higher risk of LCN sacrifice (p < 0.05). Despite the advances in skull base surgery, new postoperative LCN deficits still represent a challenge. The morbidity associated with resection of the LCNs is dependent on the tumor's size and intradural tumor extension. Though no recovery of LCN deficits may be expected, on long-term follow-up, patients usually compensate well for their LCNs loss.

  18. Three-dimensional isotropic T2-weighted cervical MRI at 3 T: Comparison with two-dimensional T2-weighted sequences

    International Nuclear Information System (INIS)

    Kwon, J.W.; Yoon, Y.C.; Choi, S.-H.

    2012-01-01

    Aim: To compare three-dimensional (3D) isotropic T2-weighted magnetic resonance imaging (MRI) sequences and reformation with two-dimensional (2D) T2-weighted sequences regarding image quality of the cervical spine at 3 T. Materials and methods: A phantom study was performed using a water-filled cylinder. The signal-to-noise and image homogeneity were evaluated. Fourteen (n = 14) volunteers were examined at 3 T using 3D isotropic T2-weighted sagittal and conventional 2D T2-weighted sagittal, axial, and oblique sagittal MRI. Multiplanar reformation (MPR) of the 3D T2-weighted sagittal dataset was performed simultaneously with image evaluation. In addition to artefact assessment, the visibility of anatomical structures in the 3D and 2D sequences was qualitatively assessed by two radiologists independently. Cohen’s kappa and Wilcoxon signed rank test were used for the statistical analysis. Result: The 3D isotropic T2-weighted sequence resulted in the highest signal-to-noise ratio (SNR) and lowest non-uniformity (NU) among the sequences in the phantom study. Quantitative evaluation revealed lower NU values of the cerebrospinal fluid (CSF) and muscles in 2D T2-weighted sagittal sequences compared to the 3D volume isotropic turbo spin-echo acquisition (VISTA) sequence. The other NU values revealed no statistically significant difference between the 2D turbo spin-echo (TSE) and 3D VISTA sequences (0.059 < p < 0.959). 3D VISTA images showed significantly fewer CSF flow artefacts (p < 0.001) and better delineated intradural nerve rootlets (p = 0.001) and neural foramina (p = 0.016) compared to 2D sequences. Conclusion: A 3D T2 weighted sequence is superior to conventional 2D sequences for the delineation of intradural nerve rootlets and neural foramina and is less affected by CSF flow artefacts.

  19. Outcome of endovascular treatment in symptomatic intracranial vascular stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Suh, Dae Chul; Kim, Sang Joon; Lee, Duk Hee; Kim, Won; Choi, Choong Gon; Lee, Jeong Hyun; Kwon, Sun Uck; Kim, Jong Sung [Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of); Kim, Hyun Jeong [Daejeon Catholic Hospital, Daejeon (Korea, Republic of)

    2005-03-15

    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.

  20. 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.

  1. Reconstruction of palatal defect using mucoperiosteal hinge flap and pushback palatoplasty.

    Science.gov (United States)

    Lee, S I; Lee, H S; Hwang, K

    2001-11-01

    This article describes a simple, new surgical technique to provide a complete two-layer closure of palatal defect resulting from a surgical complication of trans palatal resection of skull base chordoma. The nasal layer was reconstructed with triangular shape oral mucoperiosteal turn over hinge flap based on anterior margin of palatal defect and rectangular shaped lateral nasal mucosal hinge flaps. The oral layer was reconstructed with conventional pushback V-Y advancement 2-flaps palatoplasty. Each layer of the flaps were secured with two key mattress suture for flap coaptation. This technique has some advantages: simple, short operation time, one-stage procedure, no need of osteotomy. It can close small- to medium-sized palatal defect of palate or wide cleft palate and can prevent common complication of oronasal fistula, which could be caused by tension.

  2. 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.

  3. 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.

  4. Computed tomography of the nasopharynx and related spaces. Part II: pathology

    International Nuclear Information System (INIS)

    Silver, A.J.; Mawad, M.E.; Hilal, S.K.; Sane, P.; Ganti, S.R.

    1983-01-01

    Malignant tumors of the nasopharynx were analyzed by region of origin and route of spread. Nasopharyngeal carcinomas produced early submucosal infiltration of the deglutitional muscle layer with enlargement of the levator palati muscle and lateral displacement of the parapharyngeal space. Serious otitis media was frequently associated, and the trigeminal nerve was occasionally involved. Intracranial extension via the foramen lacerum was frequent. Metastases to the infratemporal fossa produced early involvement of the masticatory muscle layer with medial displacement of the parapharygeal space. Adenoid cystic carcinomas showed late but disproportionate involvement of the sphenoid sinus. Chordomas extended into the retropharyngeal soft tissues via the petro-occipital fissure. Maxillary sinus carcinomas, which were very large at the time of presentation and impinged on the nasopharynx, showed extensive destruction of the pterygoid plates

  5. Stereotactic radiosurgery using the gamma knife

    Energy Technology Data Exchange (ETDEWEB)

    Kawamoto, Shunsuke; Sasaki, Tomio; Matsutani, Masao; Takakura, Kintomo; Terahara, Atsuro (Tokyo Univ. (Japan). Faculty of Medicine)

    1992-03-01

    Since stereotactic radiosurgery using a gamma knife was developed in 1968 by Leksell, it has been used with increasing frequency in Japan. During the period from June 19, 1990 through December 20, 1991, 218 patients have been treated with stereotactic radiosurgery using a gamma knife. Of them, 116 had vascular lesions (116), including arteriovenous malformation (114), dural arteriovenous malformation (one), and cerebral aneurysm (one); and the other 102 had tumorous lesions, including acoustic neurinoma (48), meningioma (26), pituitary tumor (11), metastatic tumor (7), germ cell tumor (3), glioma (2), hemangioblastoma (2), chordoma (one), craniopharyngioma (one), and trigeminal neurinoma (one). In this article, candidates of stereotactic radiosurgery using a gamma knife are discussed, with particular attention to clinical results of the aforementioned 218 patients. (N.K.) 54 refs.

  6. Arrested Pneumatization of the Sphenoid Sinus on Large Field-of-View Cone Beam Computed Tomography Studies

    Directory of Open Access Journals (Sweden)

    Mehrnaz Tahmasbi-Arashlow

    2015-05-01

    Full Text Available Arrested pneumatization of the sphenoid sinus is a normal anatomical variant. The aim of this report is to define cone beam computed tomography (CBCT characteristics of arrested pneumatization of sphenoid sinus in an effort to help differentiate it from invasive or lytic skull base lesions. Two cases are presented with incidental findings. Both studies, acquired for other diagnostic purposes, demonstrated unique osseous patterns that were eventually deemed to be anatomic variations in the absence of clinical signs and symptoms although the pattern of bone loss and remodeling was diagnosed as pneumatization of the sphenoid sinus by a panel of medical and maxillofacial radiologists following contrasted advanced imaging. It is important to differentiate arrested pneumatization of the sphenoid sinus from lesions, such as arachnoid granulations, acoustic neuroma, glioma, metastatic lesions, meningioma, or chordoma, to prevent unnecessary biopsies or exploratory surgeries that would consequently reduce treatment costs and alleviate anxiety in patients.

  7. 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).

  8. Molecular pathology of bone tumours: diagnostic implications.

    Science.gov (United States)

    Puls, Florian; Niblett, Angela J; Mangham, D Chas

    2014-03-01

    Alongside histomorphology and immunohistochemistry, molecular pathology is now established as one of the cornerstones in the tissue diagnosis of bone tumours. We describe the principal molecular pathological techniques employed, and each of the bone tumour entities where their identified characteristic molecular pathological changes can be detected to support and confirm the suspected histological diagnosis. Tumours discussed include fibrous dysplasia, classical and subtype osteosarcomas, central and surface cartilaginous tumours, Ewing's sarcoma, vascular tumours, aneurysmal bone cyst, chordoma, myoepithelioma, and angiomatoid fibrous histiocytoma. This is a rapidly evolving field with discoveries occurring every few months, and some of the newer entities (the Ewing's-like sarcomas), which are principally identified by their molecular pathology characteristics, are discussed. © 2013 John Wiley & Sons Ltd.

  9. Delayed complications of radiotherapy treatment for nasopharyngeal carcinoma: imaging findings

    International Nuclear Information System (INIS)

    King, A.D.; Ahuja, A.T.; Yeung, D.K.; Wong, J.K.T.; Lee, Y.Y.P.; Lam, W.W.M.; Ho, S.S.M.; Yu, S.C.H.; Leung, S.-F.

    2007-01-01

    Radiotherapy is used to treat a wide variety of head and neck tumours that arise in and around the skull base. The delayed effects of radiation damages a range of structures, including the nervous system, bone, major vessels, mucus membranes, pituitary and salivary glands, as well as increasing the risk of radiation-induced neoplasms. In this review the complications resulting from radiation treatment for nasopharyngeal carcinoma (NPC), a cancer treated with a high dose of radiation to a fairly large region, are illustrated. Many patients with NPC have a long-term survival, so are at risk of developing delayed radiation effects, and hence may demonstrate a wide range of complications on imaging. Other tumours around the skull base treated with radiotherapy include meningiomas, chordomas, chondrosarcomas, pituitary adenomas, paranasal sinus and nasal cavity tumours. In these cases similar complications may be encountered on imaging, although the severity, incidence and location will vary

  10. 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)

  11. 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....

  12. Proposed Diagnostic Criteria, Classification Schema, and Review of Literature of Notochord-Derived Ecchordosis Physaliphora

    Science.gov (United States)

    Lagman, Carlito; Sarmiento, J. Manuel; Turtz, Alan R; Chitale, Rohan V

    2016-01-01

    Ecchordosis physaliphora (EP) is a benign notochordal remnant derived from ectopic nests found along the craniospinal axis. It typically presents asymptomatically and is diagnosed using classic radiologic features, particularly location, T1-hypointensity, T2-hyperintensity, and lack of enhancement following gadolinium (Gd) contrast administration. Distinguishing EP from its malignant counterpart, chordoma, is of paramount importance, given the aggressive nature of the latter. Advances in imaging and immunohistochemistry have aided in diagnosis to an extent but, to our knowledge, identification of the genetic fingerprint of EP has yet to take place. Further cytological analysis of these lesions in search of a genetic link is warranted. We propose here a set of diagnostic criteria based on features consistently cited in the literature. In this literature review, 23 case reports were identified and collated into a summary of symptomatic cases of ecchordosis physaliphora. An illustrative case report of two patients was also included.  PMID:27158576

  13. Locally challenging osteo- and chondrogenic tumors of the axial skeleton: results of combined proton and photon radiation therapy using three-dimensional treatment planning

    Energy Technology Data Exchange (ETDEWEB)

    Hug, Eugen B; Fitzek, Markus M; Liebsch, Norbert J; Munzenrider, John E

    1995-02-01

    Purpose: Tumors of the axial skeleton are at high risk for local failure. Total surgical resection is rarely possible. Critical normal tissues limit the efficacy of conventional photon therapy. This study reviews our experience of using combined high dose proton and photon radiation therapy following three-dimensional (3D) treatment planning. Methods and Materials: Between December 1980 and September 1992, 47 patients were treated at the Massachusetts General Hospital and Harvard Cyclotron Laboratory for primary or recurrent chordomas and chondrosarcomas (group 1, 20 patients), osteogenic sarcomas (group 2, 15 patients) and giant cell tumors, osteo- or chondroblastomas (group 3, 12 patients). Radiation treatment was given postoperatively in 23 patients, pre- and postoperatively in 17 patients, and 7 patients received radiation therapy as definitive treatment modality following biopsy only. The proton radiation component was delivered using a 160 MeV proton beam and the photon component using megavoltage photons up to 23 MV energy with 1.8-2.0 Cobalt Gray Equivalent (CGE) per fraction, once a day. Total external beam target dose ranged from 55.3 CGE to 82.0 CGE with mean target doses of 73.9 CGE (group 1), 69.8 CGE (group 2), and 61.8 CGE (group 3). Results: Group 1 (chordoma and chondrosarcoma): Five of 14 patients (36%) with chordoma recurred locally, and 2 out of 5 patients developed distant metastasis, resulting in 1 death from disease. A trend for improved local control was noted for primary vs. recurrent tumors, target doses > 77 CGE and gross total resection. All patients with chondrosarcoma achieved and maintained local control and disease-free status. Five-year actuarial local control and overall survival rates were 53% and 50% for chordomas and 100% and 100% for chondrosarcomas, respectively. Group 2 (osteogenic sarcoma): Three of 15 patients (20%) never achieved local control and died within 6 months of completion of radiation treatment. Only 1 out of 12

  14. The incidentaloma of the pituitary gland: Is neurosurgery required

    Energy Technology Data Exchange (ETDEWEB)

    Reincke, M.; Allolio, B.; Saeger, W.; Menzel, J.; Winkelmann, W. (Univ. of Cologne (West Germany))

    1990-05-23

    The authors describe a series of 18 patients with an intrasellar mass incidentally discovered by computed tomography or magnetic resonance imaging. The average size of the mass was 13 mm, with a range from 5 to 25 mm. Initial ophthalmologic examination revealed bitemporal hemianopia in 2 patients. Results of routine endocrine testing showed partial hypopituitarism in 5 patients and growth hormone hypersecretion without signs and symptoms of acromegaly in 1 patient. Four patients underwent neurosurgery. Histologically, one chondroid chordoma and three pituitary adenomas were found. In the remaining 14 patients treated conservatively, repeated computed tomography and magnetic resonance imaging revealed no significant change in tumor size at the time of follow-up. The results suggest that the incidentaloma of the pituitary gland is a benign condition that does not necessarily require neurosurgical intervention.

  15. 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.

  16. Imaging of sacral tumours

    International Nuclear Information System (INIS)

    Gerber, S.; Ollivier, L.; Brisse, H.; Neuenschwander, S.; Leclere, J.; Vanel, D.; Missenard, G.; Pinieux, G. de

    2008-01-01

    All components of the sacrum (bone, cartilage, bone marrow, meninges, nerves, notochord remnants, etc.) can give rise to benign or malignant tumours. Bone metastases and intraosseous sites of haematological malignancies, lymphoma and multiple myeloma are the most frequent aetiologies, while primary bone tumours and meningeal or nerve tumours are less common. Some histological types have a predilection for the sacrum, especially chordoma and giant cell tumour. Clinical signs are usually minor, and sacral tumours are often discovered in the context of nerve root or pelvic organ compression. The roles of conventional radiology, CT and MRI are described and compared with the histological features of the main tumours. The impact of imaging on treatment decisions and follow-up is also reviewed. (orig.)

  17. The incidentaloma of the pituitary gland: Is neurosurgery required?

    International Nuclear Information System (INIS)

    Reincke, M.; Allolio, B.; Saeger, W.; Menzel, J.; Winkelmann, W.

    1990-01-01

    The authors describe a series of 18 patients with an intrasellar mass incidentally discovered by computed tomography or magnetic resonance imaging. The average size of the mass was 13 mm, with a range from 5 to 25 mm. Initial ophthalmologic examination revealed bitemporal hemianopia in 2 patients. Results of routine endocrine testing showed partial hypopituitarism in 5 patients and growth hormone hypersecretion without signs and symptoms of acromegaly in 1 patient. Four patients underwent neurosurgery. Histologically, one chondroid chordoma and three pituitary adenomas were found. In the remaining 14 patients treated conservatively, repeated computed tomography and magnetic resonance imaging revealed no significant change in tumor size at the time of follow-up. The results suggest that the incidentaloma of the pituitary gland is a benign condition that does not necessarily require neurosurgical intervention

  18. Imaging of sacral tumours

    Energy Technology Data Exchange (ETDEWEB)

    Gerber, S.; Ollivier, L.; Brisse, H.; Neuenschwander, S. [Institut Curie, Department of Radiology, Paris (France); Leclere, J. [Institut Gustave Roussy, Department of Radiology, Villejuif (France); Vanel, D. [The Rizzoli Institute, Department of Radiology, Bologna (Italy); Missenard, G. [Institut Gustave Roussy, Comite de pathologie tumorale de l' appareil locomoteur, Villejuif (France); Pinieux, G. de [CHRU de Tours, Department of Pathology, Hopital Trousseau, Tours (France)

    2008-04-15

    All components of the sacrum (bone, cartilage, bone marrow, meninges, nerves, notochord remnants, etc.) can give rise to benign or malignant tumours. Bone metastases and intraosseous sites of haematological malignancies, lymphoma and multiple myeloma are the most frequent aetiologies, while primary bone tumours and meningeal or nerve tumours are less common. Some histological types have a predilection for the sacrum, especially chordoma and giant cell tumour. Clinical signs are usually minor, and sacral tumours are often discovered in the context of nerve root or pelvic organ compression. The roles of conventional radiology, CT and MRI are described and compared with the histological features of the main tumours. The impact of imaging on treatment decisions and follow-up is also reviewed. (orig.)

  19. Heidelberg Ion Therapy Center (HIT): Initial clinical experience in the first 80 patients

    Energy Technology Data Exchange (ETDEWEB)

    Combs, Stephanie E. (Univ. Hospital of Heidelberg, Dept. of Radiation Oncology, Heidelberg (Germany)), E-mail: Stephanie.Combs@med.uni-heidelberg.de; Ellerbrock, Malte; Haberer, Thomas (Heidelberger Ionenstrahl Therapiezentrum (HIT), Im Neuenheimer Feld 450, 69120 Heidelberg (Germany)) (and others)

    2010-10-15

    The Heidelberg Ion Therapy Center (HIT) started clinical operation in November 2009. In this report we present the first 80 patients treated with proton and carbon ion radiotherapy and describe patient selection, treatment planning and daily treatment for different indications. Patients and methods. Between November 15, 2009 and April 15, 2010, 80 patients were treated at the Heidelberg Ion Therapy Center (HIT) with carbon ion and proton radiotherapy. Main treated indications consisted of skull base chordoma (n = 9) and chondrosarcoma (n = 18), malignant salivary gland tumors (n=29), chordomas of the sacrum (n = 5), low grade glioma (n=3), primary and recurrent malignant astrocytoma and glioblastoma (n=7) and well as osteosarcoma (n = 3). Of these patients, four pediatric patients aged under 18 years were treated. Results. All patients were treated using the intensity-modulated rasterscanning technique. Seventy-six patients were treated with carbon ions (95%), and four patients were treated with protons. In all patients x-ray imaging was performed prior to each fraction. Treatment concepts were based on the initial experiences with carbon ion therapy at the Gesellschaft fuer Schwerionenforschung (GSI) including carbon-only treatments and carbon-boost treatments with photon-IMRT. The average time per fraction in the treatment room per patient was 29 minutes; for irradiation only, the mean time including all patients was 16 minutes. Position verification was performed prior to every treatment fraction with orthogonal x-ray imaging. Conclusion. Particle therapy could be included successfully into the clinical routine at the Dept. of Radiation Oncology in Heidelberg. Numerous clinical trials will subsequently be initiated to precisely define the role of proton and carbon ion radiotherapy in radiation oncology.

  20. L5 vertebrectomy for the surgical treatment of tumoral and traumatic lesions of L5 vertebra

    Directory of Open Access Journals (Sweden)

    Tuncay Kaner

    2012-02-01

    Full Text Available We retrospectively reviewed the clinical characteristics and the surgical results of seven patients treated with L5 vertebrectomy. The pathologies, clinical characteristics, preoperative and postoperative radiological findings, surgical techniques, and instrumentation for seven patients operated on between 1998 and 2009 are presented in this article. Biopsies were performed on all patients except those involving trauma. Patients were followed up at three-month intervals in the first year, at 6- month intervals in the second year, and on a regular basis afterward. One patient had a traumatic L5 burst fracture; the other six had tumoral pathologies in the L5 vertebrae. One tumoral lesion was a chordoma, another was a hemangioma, and the remaining four were metastatic lesions. Radiotherapy and chemotherapy were performed for the metastatic tumor patients during the postoperative period. Patients with renal cancer and chordoma survived for 3 years; patients with lung cancer and bladder cancer survived for 1 year; and patients with breast cancer survived for 16 months. The lumbosacral region presents significant stabilization problems because of the presence of sacral slope. In our opinion, if the lesion involves only the L5 vertebra, anterior cage-filled bone cement or bone graft should be performed, as dictated by the pathology and posterior transpedicular instrumentation. If the lesion involves the L4 vertebra or the sacrum and the L5 vertebra, the instrumentation can be extended to cover other segments with sacral attachments. The present cases involved only L5 vertebra and treatment with short-segment stabilization covering the anterior and posterior columns.

  1. The Radiation Response of Sarcomas by Histologic Subtypes: A Review With Special Emphasis Given to Results Achieved With Razoxane

    Directory of Open Access Journals (Sweden)

    2006-01-01

    Full Text Available Purpose. Relatively few results are available in the literature about the radiation response of unresectable sarcomas in relation to their histology. Therefore, an attempt was made to summarize the present situation. Materials and methods. This report is based on a review of the literature and the author's own experience. Adult-type soft tissue sarcomas, chondrosarcomas, and chordomas were analyzed. Radioresponse was mainly associated with the degree of tumor shrinkage, that is, objective responses. Histopathologic responses, that is, the degree of necrosis, are only discussed in relation to radiation treatment reports of soft tissue sarcomas as a group. Results. Radiation therapy alone leads to major responses in about 50% of lipo-, fibro-, leiomyo-, or chondrosarcomas. The response rate is less than 50% in malignant fibrous histiocytomas, synovial, neurogenic, and other rare soft tissue sarcomas. The response rates may increase up to 75% through the addition of radiosensitizers such as halogenated pyrimidines or razoxane, or by the use of high-LET irradiation. Angiosarcomas become clearly more responsive if biologicals, angiomodulating, and/or tubulin affinic substances are given together with radiation therapy. Razoxane is able to increase the duration and quality of responses even in difficult-to-treat tumors like chondrosarcomas or chordomas. Conclusions. The available data demonstrate that the radioresponsiveness of sarcomas is very variable and dependent on histology, kind of radiation, and various concomitantly given drugs. The rate of complete sustained remissions by radiation therapy alone or in combination with drugs is still far from satisfactory although progress has been made through the use of sensitizing agents.

  2. The progress in radiotherapy techniques and it's clinical implications

    International Nuclear Information System (INIS)

    Reinfuss, M.; Walasek, T.; Byrski, E.; Blecharz, P.

    2011-01-01

    Three modem radiotherapy techniques were introduced into clinical practice at the onset of the 21 st century - stereotactic radiation therapy (SRT), proton therapy and carbon-ion radiotherapy. Our paper summarizes the basic principles of physics, as well as the technical reqirements and clinical indications for those techniques. SRT is applied for intracranial diseases (arteriovenous malformations, acoustic nerve neuromas, brain metastases, skull base tumors) and in such cases it is referred to as stereotactic radiosurgery (SRS). Techniques used during SRS include GammaKnife, CyberKnife and dedicated linacs. SRT can also be applied for extracranial disease (non-small cell lung cancer, lung metastases, spinal and perispinal tumors, primary liver tumors, breast cancer, pancreatic tumors, prostate cancer, head and neck tumors) and in such cases it is referred to as stereotactic body radiation therapy (SBRT). Eye melanomas, skull base and cervical spine chordomas and chordosarcomas, as well as childhood neoplasms, are considered to be the classic indications for proton therapy. Clinical trials are currently conducted to investigate the usefulness of proton beam in therapy of non-small cell lung cancer, prostate cancer, head and neck tumors, primary liver and oesophageal cancer Carbon-ion radiotherapy is presumed to be more advantageous than proton therapy because of its higher relative biological effectiveness (RBE) and possibility of real-time control of the irradiated volume under PET visualization. The basic indications for carbon-ion therapy are salivary glands neoplasms, selected types of soft tissue and bone sarcomas, skull base chordomas and chordosarcomas, paranasal sinus neoplasms, primary liver cancers and inoperable rectal adenocarcinoma recurrences. (authors)

  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. Leakage detection on CT myelography for targeted epidural blood patch in spontaneous cerebrospinal fluid leaks: calcified or ossified spinal lesions ventral to the thecal sac.

    Science.gov (United States)

    Yoshida, Hiroki; Takai, Keisuke; Taniguchi, Makoto

    2014-09-01

    The purpose of this study was to describe significant CT myelography findings for determination of the leak site and outcome of targeted epidural blood patch (EBP) in patients with spontaneous CSF leaks. During 2005-2013, spontaneous CSF leaks were diagnosed for 12 patients with orthostatic headaches. The patients received targeted EBP on the basis of CT myelography assessments. Computed tomography myelograms revealed ventral extradural collection of contrast medium distributed over multiple spinal levels (average 16 levels). Intraforaminal contrast medium extravasations were observed at multiple spinal levels (average 8.2 levels). For 8 (67%) of 12 patients, spinal lesions were noted around the thecal sac and included calcified discs with osteophytes, an ossified posterior longitudinal ligament, and an ossified yellow ligament; lesions were mostly located ventral to the thecal sac and were in close contact with the dura mater. The levels of these spinal lesions were considered potential leak sites and were targeted for EBP. For the remaining 4 patients who did not have definite spinal lesions around the thecal sac, leak site determination was based primarily on the contrast gradient hypothesis. The authors hypothesized that the concentration of extradural contrast medium would be the greatest and the same as that of intradural contrast medium at the leak site but that it would decrease with increased distance from the leak site according to the contrast gradient. Epidural blood patch was placed at the level of spinal lesions and/or of the greatest and same concentration of contrast medium between the intradural and extradural spaces. For 10 of the 12 patients, the orthostatic headaches decreased significantly within a week of EBP and disappeared within a month. For the remaining 2 patients, headaches persisted and medical treatment was required for several months. For 3 patients, thick chronic subdural hematomas caused severe headaches and/or disturbed

  5. 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

  6. 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

  7. 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

  8. 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

  9. 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

  10. Somatostatin receptor imaging in intracranial tumours

    Energy Technology Data Exchange (ETDEWEB)

    Schmidt, M.; Scheidhauer, K.; Voth, E.; Schicha, H. [Department of Nuclear Medicine, University of Koeln (Germany); Luyken, C.; Hildebrandt, G.; Klug, N. [Department of Neurosurgery, University of Kolen (Germany)

    1998-07-01

    The somatostatin analogue [{sup 111}In-DTPA-d-Phe{sup 1}]-octreotide ({sup 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 {sup 111}In-octreotide scintigraphy has a clearly defined role in various neuroendocrine diseases, the clinical value of {sup 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), {sup 111}In-octreotide scintigraphy was performed 4-6 and 24 h after i.v. injection of 110-220 MBq {sup 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 {sup 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 {sup 111}In-octreotide uptake. The results demonstrate that a large variety of intracranial

  11. 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.)

  12. 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)

  13. 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.)

  14. 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.)

  15. Transcranial and Epidural Approach for Spontaneous Cerebrospinal Fluid Leakage Due to Meningoencephalocele of the Lateral Sphenoid Sinus.

    Science.gov (United States)

    Shintoku, Ryosuke; Tosaka, Masahiko; Shimizu, Tatsuya; Yoshimoto, Yuhei

    2018-01-01

    We experienced a case of sphenoid sinus type meningoencephalocele manifesting as severe cerebrospinal fluid (CSF) rhinorrhea. A 35-year-old man became aware of serous nasal discharge 1 year previously, which had gradually worsened. The nasal discharge was diagnosed as CSF rhinorrhea. Head computed tomography (CT) showed several small depressions in the bone of the left middle cranial fossa, and the largest depression extended through the bone to the lateral sphenoid sinus. Head magnetic resonance imaging revealed that the meningoencephalocele projected to the lateral sphenoid sinus, through this small bone defect of the middle cranial fossa. We performed a combined craniotomy and epidural approach without intradural procedures using neuronavigation. Multiple meningoencephaloceles protruded into small depressions in the middle skull base. The small protrusions not passing through the sphenoid sinus were coagulated. The largest protrusion causing the CSF leakage was identified by neuronavigation. This meningoencephalocele was cut. Both the dural and bone sides were closed with double layers to prevent CSF leakage. The CSF rhinorrhea completely stopped after the surgery. In our case, identification of the leak site was easy with neuronavigation based on bone window CT. The epidural approach also has significant advantages with double layer closure, including both the dural and bone sides. If the site of CSF leakage is outside the foramen rotundum (as with the most common type of lateral sphenoid sinus meningoencephalocele), we recommend the epidural approach using neuronavigation for surgical treatment.

  16. Quantitative study of the opticocarotid and carotid-oculomotor windows for the interpeduncular fossa, before and after internal carotid artery mobilization and posterior communicating division.

    Science.gov (United States)

    Kim, Young-Don; Elhadi, Ali M; Mendes, George A C; Maramreddy, Naveen; Agrawal, Abhishek; Kalb, Samuel; Nakaji, Peter; Spetzler, Robert F; Preul, Mark C

    2015-03-01

    The management of basilar apex (BX) aneurysms remains problematic. We quantified the surgical exposure of the BX through the opticocarotid window (OCW) and the carotid-oculomotor window (COW), before and after mobilization of the internal carotid artery and division of the posterior communicating artery (PCoA). Eleven silicone-injected cadaveric heads were dissected bilaterally. The surgical dissection was divided into 4 major steps: (1) supraorbital modified orbitozygomatic craniotomy, (2) mobilization of the internal carotid artery after drilling out the anterior clinoid process intradurally and cutting the distal dural ring, (3) drilling out the posterior clinoid process and dorsum sellae, and (4) dividing the PCoA from the posterior third portion of the vessel. A frameless navigation system was used to quantify the surgical exposure area of the BX through the OCW and COW. The total surgical area increased significantly from steps 1 to 4 (P PCoA significantly increased the overall surgical area for permanent clip application (P PCoA and height of the BX.

  17. 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.

  18. 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)

  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. 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.

  2. ROLE OF MRI IN EVALUATION OF COMPRESSIVE MYELOPATHY

    Directory of Open Access Journals (Sweden)

    Raseshkumar Rasiklal Vyas

    2017-03-01

    Full Text Available BACKGROUND Aim of the study was to find out various causes of compressive myelopathy and to characterise them. MATERIALS AND METHODS Total of 48 cases were analysed over a period of January 2016 to January 2017 and were evaluated using MRI spine studies. RESULTS MRI, because of its exemplary tissue characterisation and high contrast resolution, excellently demonstrates the anatomical details and pathological process. Thus, is a superior modality in diagnosing Spinal cord lesions as well as associated soft tissue injuries, inter-vertebral discs and ligaments. In our study, traumatic injuries (43% were found to be the most common cause of Compressive myelopathy, other were Infections (23%, primary malignancies (17%, and Metastasis (17%. Thoracic spine was found to be the most frequent site in cases of Traumatic injuries. 40 out of total 48 cases had extradural, and the rest 8 had intra-dural compressive lesions. CONCLUSION The study concludes that patients with suspected Compressive myelopathies benefit from evaluation with MRI, which is highly accurate for characterising and identifying the underlying aetiology, as well as associated features. Thus, explicitly helps in stating the long-term prognosis of the patient.

  3. Acute, Nontraumatic Spontaneous Spinal Subdural Hematoma: A Case Report and Systematic Review of the Literature

    Science.gov (United States)

    Abel, Taylor J.

    2017-01-01

    Spontaneous spinal subdural hematoma (sSDH) is a rare condition outright. Moreover, cases that occur spontaneously in the absence of an identifiable etiology are considerably less common and remain poorly understood. Here, we present the case of a 43-year-old man with spontaneous sSDH presenting with acute onset low back pain and paraplegia. Urgent magnetic resonance imaging identified a dorsal SDH from T8 to T11 with compression of the spinal cord. Emergent T8–T10 laminectomies with intradural exploration and hematoma evacuation were performed. However, despite prompt identification and appropriate action, the patient's recovery was modest and significant disability remained at discharge. This unique and unusual case demonstrates that spontaneous sSDH requires prompt surgical treatment to minimize associated morbidity and supports the association between the presence of severe neurological deficits upon initial presentation with less favorable outcomes. We performed a comprehensive systematic review of spontaneous sSDH of unknown etiology, which demonstrates that emergent surgical intervention is indicated for patients presenting with severe neurological deficits and the presence of these deficits is predictive of poor neurological outcome. Furthermore, conservative management should be considered in patients presenting with mild neurological deficits as spontaneous resolution followed by favorable neurological outcomes is often observed in these patients. PMID:29441210

  4. Stroke in Ehlers-Danlos Syndrome Kyphoscoliotic Type: Dissection or Vasculitis?

    Science.gov (United States)

    Quade, Annegret; Wiesmann, Martin; Weis, Joachim; Kurth, Ingo; Jalaie, Houman; Rohrbach, Marianne; Häusler, Martin

    2017-09-01

    Patients with the kyphoscoliotic type of Ehlers-Danlos syndrome have an increased risk of vascular complications such as aortic dissection and perforation. Cerebral ischemia has only rarely been documented. This 13-year-old girl with the kyphoscoliotic type of Ehlers-Danlos syndrome experienced a large right middle cerebral artery distribution infarction. Full intravenous heparinization was started in response to presumed arterial dissection. Magnetic resonance imaging studies including magnetic resonance angiography and digital subtraction angiography, however, did not confirm dissection but suggested with cerebral vasculitis extending from the intradural right internal carotid artery to the M2 branches of the middle cerebral artery. Combined steroid and cyclophosphamide therapy was associated with clinical improvement. Two months later she died from hemorrhagic shock caused by a two-sided spontaneous rupture of the aortic artery. Cerebral vasculitis should be included in the differential diagnosis of vascular complications in kyphoscoliotic type of Ehlers-Danlos syndrome. Copyright © 2017 Elsevier Inc. All rights reserved.

  5. Kinematic analysis of the gait of adult sheep during treadmill locomotion: Parameter values, allowable total error, and potential for use in evaluating spinal cord injury.

    Science.gov (United States)

    Safayi, Sina; Jeffery, Nick D; Shivapour, Sara K; Zamanighomi, Mahdi; Zylstra, Tyler J; Bratsch-Prince, Joshua; Wilson, Saul; Reddy, Chandan G; Fredericks, Douglas C; Gillies, George T; Howard, Matthew A

    2015-11-15

    We are developing a novel intradural spinal cord (SC) stimulator designed to improve the treatment of intractable pain and the sequelae of SC injury. In-vivo ovine models of neuropathic pain and moderate SC injury are being implemented for pre-clinical evaluations of this device, to be carried out via gait analysis before and after induction of the relevant condition. We extend previous studies on other quadrupeds to extract the three-dimensional kinematics of the limbs over the gait cycle of sheep walking on a treadmill. Quantitative measures of thoracic and pelvic limb movements were obtained from 17 animals. We calculated the total-error values to define the analytical performance of our motion capture system for these kinematic variables. The post- vs. pre-injury time delay between contralateral thoracic and pelvic-limb steps for normal and SC-injured sheep increased by ~24s over 100 steps. The pelvic limb hoof velocity during swing phase decreased, while range of pelvic hoof elevation and distance between lateral pelvic hoof placements increased after SC injury. The kinematics measures in a single SC-injured sheep can be objectively defined as changed from the corresponding pre-injury values, implying utility of this method to assess new neuromodulation strategies for specific deficits exhibited by an individual. Copyright © 2015 Elsevier B.V. All rights reserved.

  6. [Airway management in a man with ankylosing spondylitis].

    Science.gov (United States)

    Gil, S; Jamart, V; Borrás, R; Miranda, A

    2007-02-01

    We report a case of difficult airway management in a 41-year-old man with ankylosing spondylitis who was scheduled for total left hip replacement surgery. After several failed attempts to achieve regional anesthesia, we converted to general anesthesia with orotracheal intubation using a fiberoptic bronchoscope. Ankylosing spondylitis leads to fibrosis, ossification, and ankylosis along the spinal column and sacroiliac articulations. Cervical column and atlantooccipital articulation mobility are reduced and in severe cases the cervical vertebrae become fixed in a flexed position. This portion of the spine is also the most susceptible to fracture, particularly in hyperextension, an event that could lead to damage to the cervical spinal cord during maneuvers to manage the airway. Patients with this condition may also have temporomandibular joint involvement, further complicating airway management. We report the case of a patient with ankylosing spondylitis with fixation along the entire spine. The airway was managed by intubation with a fiberoptic bronchoscope. Spontaneous ventilation was maintained during the maneuver, and sedation was achieved with perfusion of remifentanil as the only anesthetic agent following failure of intradural anesthesia.

  7. Spinal dermoid cyst

    International Nuclear Information System (INIS)

    Miyamoto, Yoshihisa; Makita, Yasumasa; Nabeshima, Sachio; Tei, Taikyoku; Keyaki, Atsushi; Takahashi, Jun; Kawamura, Junichiro

    1987-01-01

    A 25-year-old male complained of intermittent, sharp pains about the left eye and in the left side of the chest. Neurological examination revealed paresthesia and impaired perception of touch and pin-pricks in the dermatomes of Th8 and Th9 on the left side. In all four extremities, the muscle stretch reflexes were equal and slightly hyperactive, without weakness or sensory deficits. Metrizamide myelography showed defective filling at the level between the upper 8th and 9th thoracic vertebrae. The lesion was also demonstrated by computed tomography (CT) scan performed 1 hour later, appearing as an oval, radiolucent mass in the left dorsal spinal canal, which compressed the spinal cord forward and toward the right. Serial sections of the spinal canal revealed the lesion to be partly filled with contrast medium. Repeat CT scan 24 hours after metrizamide myelography showed more contrast medium in the periphery of the lesion, giving it a doughnut-shaped appearance. At surgery a smooth-surfaced cyst containing sebum and white hair was totally removed from the intradural extramedullary space. The histological diagnosis was dermoid cyst. There have been a few reported cases of intracranial epidermoid cyst in which filling of the cyst was suggested on metrizamide CT myelography. These findings may complicate the differential diagnosis of arachnoid cyst and dermoid or epidermoid cyst when only CT is used. (author)

  8. Complete rectal prolapse clinical and functional outcome with Delorme's procedure: Resultados clínicos y funcionales con la operación de Delorme Prolapso rectal completo

    Directory of Open Access Journals (Sweden)

    J. A. Pascual Montero

    2006-11-01

    Full Text Available Introduction: many surgical techniques -both through the perineal and abdominal routes- have been described for the treatment of rectal prolapse. The aim of this work is to evaluate the clinical and functional outcome with Delorme's perineal procedure. Patients and methods: twenty-one patients with complete rectal prolapse were studied from July 2000 to October 2005. Age, gender, anesthetic risk, and accompanying symptoms were all assessed. Diagnostic tests performed included: colonoscopy, anorectal manometry before and after surgery, and 360° endoanal ultrasonography. Delorme's procedures were carried out by only one surgical team. Results: no mortality occurred, and morbidity was minimal. Prolapse relapse rate was 9.52% with a mean follow-up of 34 months. Anal continence improved in 87.5% of patients, and no surgery-associated constipation ensued. Mean hospital stay was 2 (range 1-4 days. During the postoperative period no pain developed in 17 patients, and 4 patients had mild pain. Satisfaction with surgery was high in 16 cases (76.19%, moderate in 3 (14.28%, and low in 2 (9.52%. Conclusions: Delorme's procedure for the management of complete rectal prolapse is associated with low morbidity, improves anal continence, gives rise to no postsurgical constipation, and has an acceptable relapse rate. Patient satisfaction with this procedure is high because of its high comfortability (intradural anesthesia, short hospital stay, and little postoperative pain and optimal results.

  9. 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.

  10. 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.

  11. 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)

  12. Transcavernous Approach to the Basilar Apex: A Cadaveric Prosection

    Science.gov (United States)

    Sellin, Jonathan N; Navarro, Jovany C; Batjer, Hunt H; Van Loveren, Harry; Duckworth, Edward A

    2018-01-01

    The transcavernous approach to the basilar artery, as initially described by Dolenc, is one of the most common and elegant approaches to the region. It affords a generous working and viewing angle, but it can be technically challenging and requires attention to detail at each step. We investigate this approach in this report via a cadaveric prosection with a focus on the value of each of the component steps in improving surgical view and exposure. The transcavernous approach steps are divided into extradural stages: orbitozygomatic osteotomy (a modern adjunct to Dolenc’s original description), drilling of the lesser sphenoid wing, and anterior clinoidectomy; and intradural stages: wide splitting of the Sylvian fissure, unroofing of the oculomotor and trochlear nerves, and posterior clinoidectomy. The surgical windows afforded by each step in the approach are illustrated using microscopic images taken during the cadaveric prosection of a donor who happened to harbor a basilar apex aneurysm. An illustrative case and artist illustrations are used to emphasize the relative value of each step of the transcavernous exposure. PMID:29682431

  13. A Large Asymptomatic Thoracic Spine Schwannoma Detected Incidentally by {sup 18}F-FDG PET/CT: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Bahk, Yong Whee [Seoul Sungae Hospital, Seoul (Korea, Republic of); Yoo, Ie Ryung [Catholic University Medical School Hospital, Seoul (Korea, Republic of)

    2008-10-15

    Schwannoma is not a rare tumor occurring anywhere where sheathed nerve fibers present. However the spinal involvement has been noted to be uncommon. The use of PET in the diagnosis of spinal schwannomas seemed to have only sporadically been reported and standardized uptake values (SUV) measured in peripheral nerves schwannomas varied according to cellularity. Most reported spinal schwannoms were symptomatic and relatively hypometabolic but ours differed in that despite considerable compression of the spinal cord it did not produce clinical symptoms or signs and had a relatively high FDG uptake value. The spine was operated. On opening a 1.0x1.5 cm tumor was found to be encapsulated and located in the right posterolateral aspect of the intradural space at the 4th thoracic spine level. It was completely resected along with the rootlet from which the tumor seemed to have originated not damaging the cord or causing bleeding. The spinal medulla compressed by and adherent to the tumor was decompressed and released following arachnoid adhesiolysis. The hospital stay was uneventful. Patient was discharged and is well and fine without complication eight months after surgery.

  14. [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.

  15. 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)

  16. Hydatid disease of the spine: A rare case

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    Mona Agnihotri

    2017-01-01

    Full Text Available Hydatid disease or hydatidosis is the most widespread zoonosis caused by Echinococcus granulosus. Liver and lungs are the most common sites. Bone involvement is rare and reported in 0.5%–4% with spinal involvement reported in 50% of these cases. We present a case of spinal hydatidosis in a 35-year-old male presenting with lower extremity weakness and numbness. Magnetic resonance imaging (MRI of the spine showed multiple cystic lesions at the T9–T11 level with involvement of the paraspinal muscles. The lesion was seen intraspinal, intradural, intramedullary, and epidural. Radiological impression was aneurysmal bone cyst. The patient underwent laminectomy, and the excised cysts showed characteristic features of hydatid cyst (HC on histopathology. The patient was started on antihelminthic therapy postoperatively. MRI is a diagnostic modality for HC, but the unusual location and absence of characteristic features can cause diagnostic difficulty. A high index of suspicion should be kept in patients residing in endemic areas and presenting with unusual cystic lesion of spine.

  17. 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.)

  18. Spinal subspecialization in post-graduate neurosurgical education.

    Science.gov (United States)

    Toyota, Brian D

    2004-05-01

    The growing science and technology of various neurosurgical areas fosters subspecialization. The transmission of this expanding knowledge base to the neurosurgical resident becomes an increasing challenge. A survey of neurosurgical residency program directors was undertaken to evaluate their response to the budding subspecialization of spine surgery within general neurosurgery. A survey requesting background data, educational infrastructure and prevailing opinion was distributed to all 13 neurosurgical program directors in Canada. The responses were tabulated and results recorded. It is upon these results that conclusions and proposed directions are based. The current practice of the overwhelming majority of Canadian academic neurosurgical centers is to have neurosurgical spinal subspecialists working under the umbrella of the general neurosurgical division. A large percentage of neurosurgical program directors in Canada believe that the management of spinal disease, including both intradural procedures and instrumentation, is and should remain an integral part of general neurosurgical training. A consensus statement regarding the requirements of neurosurgical training in spinal disorders is the expressed desire of almost all program directors. A proposed direction and resolution is discussed.

  19. Sir Victor Horsley (1857-1916): pioneer of neurological surgery.

    Science.gov (United States)

    Tan, Tze-Ching; Black, Peter McL

    2002-03-01

    Immortalized in surgical history for the introduction of "antiseptic wax," Sir Victor Horsley played a pivotal role in shaping the face of standard neurosurgical practice. His contributions include the first laminectomy for spinal neoplasm, the first carotid ligation for cerebral aneurysm, the curved skin flap, the transcranial approach to the pituitary gland, intradural division of the trigeminal nerve root for trigeminal neuralgia, and surface marking of the cerebral cortex. A tireless scientist, he was a significant player in discovering the cure for myxedema, the eradication of rabies from England, and the invention of the Horsley-Clarke stereotactic frame. As a pathologist, Horsley performed research on bacteria and edema and founded the Journal of Pathology. Horsley's kindness, humility, and generous spirit endeared him to patients, colleagues, and students. Born to privilege, he was nonetheless dedicated to improving the lot of the common man and directed his efforts toward the suffrage of women, medical reform, and free health care for the working class. Knighted in 1902 for his many contributions to medicine, Sir Victor met an untimely death during World War I from heat stroke at the age of 59. An iconoclast of keen intellect, unlimited energy, and consummate skill, his life and work justify his epitaph as a "pioneer of neurological surgery."

  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. Huge interparietal posterior fontanel meningohydroencephalocele

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    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.

  2. Characterization of fatty acid binding by the P2 myelin protein

    International Nuclear Information System (INIS)

    Gudaitis, P.G.; Weise, M.J.

    1987-01-01

    In recent years, significant sequence homology has been found between the P2 protein of peripheral myelin and intracellular retinoid- and fatty acid-binding proteins. They have found that salt extracts of bovine intradural nerve roots contain the P2 basic protein in association with free fatty acid. Preliminary results from quantitative analyses showed a ratio of 0.4-1.1 fatty acid (mainly oleate and palmitate) per P2 molecule. P2/ligand interactions were partially characterized using ( 3 H)-oleate in gel permeation assays and binding studies using lipidex to separated bound and free fatty acid. Methyloleate was found to displace ( 3 H)-oleate from P2, indicating that ligand binding interactions are predominantly hydrophobic in nature. On the other hand, myristic acid and retinol did not inhibit the binding of oleate to the protein, results consistent with a decided affinity for long chain fatty acids but not for the retinoids. The binding between P2 and oleic acid showed an apparent Kd in the micromolar range, a value comparable to those found for other fatty acid-binding proteins. From these results they conclude that P2 shares not only structural homology with certain fatty acid binding proteins but also an ability to bind long chain fatty acids. Although the significance of these similarities is not yet clear, they may, by analogy, expect P2 to have a role in PNS lipid metabolism

  3. 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

  4. 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.

  5. Lumbosacral lipoma : gadolinium-enhanced fat saturation T1 weighted MR image is necessary?

    International Nuclear Information System (INIS)

    Yoon, Man Won; Kim, Hyun Chul; Chung, Tae Woong; Seo, Jeong Jin; Chung, Gwang Woo; Kim, Yun Hyeon; Kim, Jae Kyu; Park, Jin Gyoon; Kang, Heoung Keun

    1999-01-01

    To evaluate the usefulness of contrast-enhanced fat saturation T1-weighted imaging for the evaluation of spinal lipoma, compared with clinical symptoms and surgical findings. Ten patients with lipomyelomeningocele, confirmed by surgery, were included in this study. In all cases, conventional spin echo T1-and T2-weighted MR imaging, and contrast-enhanced fat saturation T1-weighted imaging was performed to evaluate clinical symptoms, the position of the conus medullaris, the presence of cord tethering, and associated anomalies, and to compare the relative usefulness of the techniques. All ten patients were suffering from lipomyelomeningocele without filum terminale fibrolipoma or intradural lipoma. All cases were associated with cord tethering. As associated anomalies, there were seven cases of syringomyelia without hydrocephalus or anorectal anomaly. To evaluate the position of the spinal conus and the presence of cord tethering, conventional T1-weighted imaging was more useful than the contrast-enhanced fat saturation equivalent. In patients with early-stage spinal lipoma, MRI is useful for evaluation of the causes and position of cord tethering and associated anomalies Our results suggest that contrast-enhanced fat saturation T1-weighted images do not provide additional information concerning spinal lipoma, and that for the diagnosis of this condition, conventional T1 and T2-weighted images are more useful than those obtained by contrast-enhanced fat saturation T1-weighted imaging

  6. Thoracolumbar Langerhans cell histiocytosis in a toddler

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    Zhi Gang Lan

    2018-01-01

    Full Text Available Introduction: Langerhans cell histiocytosis (LCH is a rare uni or multisystem disorder associated with extreme production of immunoreactive Langerhans cells. Although this disorder has been reported in all age groups, spinal involvement especially thoracolumbar spine is seldom reported in toddlers. Case presentation: We present a one (1 year, four (4 months female child with a history of recurrent fever, irritability and severe anemia of one (1 month duration. CT-scan and MRI revealed a collapsed twelfth thoracic vertebra (T12 body height greater than 95% with a huge intradural soft tissue mass. T12 cortectomy via posterior thoracolumbar approach was used to decompress the soft tissue part followed by interbody fusion with titanium cage filled with autologous iliac crest bone graft, fixation using plates and screws. Conclusion: We are of the view that surgical decompression of spinal LHC lesions with interbody fusion with titanium cage filled with autologous iliac crest bone graft and fixation using plates and screws is very crucial in relieving neurological deficits. However, the patient will need repeated surgeries as she advances with age in a timely manner to avert any neurological deficit that may occur. Keywords: Langerhans cell histiocytosis (LCH, Letterer-Siwe disease, Hand-Schüller-Christian disease, Eosinophilic granuloma

  7. Pediatric spinal infections

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    Raj Kumar

    2014-01-01

    Full Text Available The infections of the spinal axis in children are rare when compared with adults. They encompass a large spectrum of diseases ranging from relatively benign diskitis to spinal osteomyleitis and to the rapidly progressive, rare, and potentially devastating spinal epidural, subdural, and intramedullary spinal cord infections. We present a comprehensive review of the literature pertaining to these uncommon entities, in light of our experience from northern India. The most prevalent pediatric spinal infection in Indian scenario is tuberculosis, where an extradural involvement is more common than intradural. The craniovertebral junction is not an uncommon site of involvement in children of our milieu. The majority of pyogenic infections of pediatric spine are associated with congenital neuro-ectodermal defects such as congenital dermal sinus. The clinico-radiological findings of various spinal infections commonly overlap. Hence the endemicity of certain pathogens should be given due consideration, while considering the differential diagnosis. However, early suspicion, rapid diagnosis, and prompt treatment are the key factors in avoiding neurological morbidity and deformity in a growing child.

  8. Evaluation of paraclinoid aneurysm by using contrast-enhanced MR angiography

    International Nuclear Information System (INIS)

    Tsuboi, Toshiyuki; Takahashi, Kazuya; Date, Isao; Mandai, Shinya; Kinugasa, Kazushi; Nishimoto, Akira

    2004-01-01

    Juxta-dural ring aneurysms of the internal carotid artery have different clinical courses and risks of subarachnoid hemorrhage (SAH) depending upon their positions in the intradural or extradural spaces. The goal of this study was to localize such aneurysms using contrast enhanced-MR Angiography (CE-MRA). CE-MRA studies were performed in 18 patients with juxta-dural ring aneurysms using three-dimensional time-of-flight spoiled gradient-recalled acquisition in the steady state (3D-TOF-SPGR) sequence. Imaging parameters were as follows: 33/3.4/1 (repetition time/echo time/number of excitation (TR/TE/NEX)), flip angle 25 deg, 256 x 192 matrix, 0.8-mm slice thickness, 60 sections in total (one slab), 180-mm field of view, with no magnetization transfer contrast (MTC), superior saturation (S sat) pulse, total imaging time 6 minutes 53 seconds. We evaluated both the source images of CE-MRA and multiplanar reconstruction (MPR) images. When the hyperintensity of the aneurysm was located within the hyperintensity of the contrast-enhanced cavernous sinus, we diagnosed the lesion as an intracavernous, ''extradural'' aneurysm. In addition, we compared CE-MRA with operative findings. Seven patients out of 18 were treated surgically. In all, MRI findings corresponded well with the operative findings. We believe CE-MRA and its MPR images using 3D-TOF-SPGR sequence is a useful technique to evaluate juxta-dural ring aneurysms. (author)

  9. Imaging in spine and spinal cord malformations

    International Nuclear Information System (INIS)

    Rossi, Andrea; Biancheri, Roberta; Cama, Armando; Piatelli, Gianluca; Ravegnani, Marcello; Tortori-Donati, Paolo

    2004-01-01

    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

  10. Temozolomide-Induced Shrinkage of a Pituitary Carcinoma Causing Cushing's Disease — Report of a Case and Literature Review

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    Lorenzo Curtò

    2010-01-01

    Full Text Available Temozolomide (TMZ is an alkylating chemotherapeutic agent that has recently been used in some cases as a new therapeutic tool for pituitary carcinomas and aggressive pituitary adenomas. In this report, we present the case of effective TMZ treatment in a 42-year-old man with ACTH-secreting carcinoma. The tumor grew progressively over 4 years, from 2.2 to 31.1 cm3, despite three surgical approaches and gamma-knife treatment. Ki-67 increased from 2 to 18%. An intradural metastasis at the foramen magnum was detected by MRI after the third operation. Thereafter, four cycles of 5-day TMZ administration (200 mg/m2/day during the first, and 150 mg/m2/day during the following cycles induced dramatic tumor size reduction (>90%. Clinical conditions improved progressively and, after 17 months from the beginning of TMZ administration, the patient is still alive. The treatment was well tolerated except for a transient thrombocytopenia (grade 4 WHO.

  11. Microsurgical anatomy of the abducens nerve.

    Science.gov (United States)

    Joo, Wonil; Yoshioka, Fumitaka; Funaki, Takeshi; Rhoton, Albert L

    2012-11-01

    The aim of this study is to demonstrate and review the detailed microsurgical anatomy of the abducens nerve and surrounding structures along its entire course and to provide its topographic measurements. Ten cadaveric heads were examined using ×3 to ×40 magnification after the arteries and veins were injected with colored silicone. Both sides of each cadaveric head were dissected using different skull base approaches to demonstrate the entire course of the abducens nerve from the pontomedullary sulcus to the lateral rectus muscle. The anatomy of the petroclival area and the cavernous sinus through which the abducens nerve passes are complex due to the high density of critically important neural and vascular structures. The abducens nerve has angulations and fixation points along its course that put the nerve at risk in many clinical situations. From a surgical viewpoint, the petrous tubercle of the petrous apex is an intraoperative landmark to avoid damage to the abducens nerve. The abducens nerve is quite different from the other nerves. No other cranial nerve has a long intradural path with angulations and fixations such as the abducens nerve in petroclival venous confluence. A precise knowledge of the relationship between the abducens nerve and surrounding structures has allowed neurosurgeon to approach the clivus, petroclival area, cavernous sinus, and superior orbital fissure without surgical complications. Copyright © 2012 Wiley Periodicals, Inc.

  12. Atypical Central Neurocytoma with Recurrent Spinal Dissemination over a Period of 20 Years: A Case Report and Review of the Literature

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    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.

  13. Preoperative trans-arterial embolization for spinal tumor: embolization techniques and results

    International Nuclear Information System (INIS)

    Shi Haibin; Xu Daizhe

    2000-01-01

    Objective: To analyze the technique and to evaluate the safety and value of preoperative trans-arterial embolization of hypervascular spinal tumors. Methods: Eighteen patients with hypervascular spinal tumors underwent trans-arterial embolization before surgery. They arose intradural in six patients and extradural in 12. Thirty-one arteries were embolized with polyvinyl alcohol (PVA) particles (150-500 μm), of which 18 with additional pieces of gelatin sponge for proximal pedicular embolization. The criteria for judging the effectiveness of embolization were the completeness of tumor removal and estimated blood loss during surgery. Results: Tumor embolization with total occlusion was obtained in eight patients, second to total in seven, subtotal in one, and partial in two. There were no symptomatic complications associated with embolization. Tumors were totally removed in 17 patients and nearly totally removed in one. The average estimated blood loss during surgery was 1100 ml (range, 200-6000 ml) for all 18 patients, and 1540 ml in patients with extradural tumors. Conclusions: Preoperative embolization of hypervascular spinal tumors is safe and effective. It can make complete resection of a tumor possible and can make an unresectable tumor resectable. Superselection or flow control is necessary to achieve effective devascularization and avoid complications

  14. The Management of Cerebrospinal Fluid Leak After Anterior Cervical Decompression Surgery.

    Science.gov (United States)

    Zhai, Jiliang; Panchal, Ripul R; Tian, Ye; Wang, Shujie; Zhao, Lijuan

    2018-03-01

    Cerebrospinal fluid (CSF) leak is a rare but potentially troublesome and occasionally catastrophic complication after anterior cervical decompression surgery. There is limited literature describing this complication, and the management of CSF leak varies. The aim of this study was to retrospectively review the treatment of cases with CSF leak and develop a management algorithm. A series of 14 patients with CSF leak from January 2011 to May 2016 were included in this study. Their characteristics, management of CSF leak, and outcomes were documented. There were 5 male and 9 female patients. Mean age at surgery was 57.1±9.9 years (range, 37-76 years). All instances of CSF leak, except 1 noted postoperatively, were indirectly repaired intraoperatively. A closed straight wound drain was placed for all patients. A lumbar subarachnoid drain was placed immediately after surgery in 4 patients and postoperatively in 7 patients. In 1 patient, lumbar drain placement was unsuccessful. In 2 additional patients, the surgeon decided not to place a lumbar drain. One patient developed meningitis and recovered after antibiotic therapy with meropenem and vancomycin. Another patient had a deep wound infection and required a revision surgery. Wound drains and lumbar drains should be immediately considered when CSF leak is identified. Antibiotics also should be considered to prevent intradural infection. [Orthopedics. 2018; 41(2):e283-e288.]. Copyright 2018, SLACK Incorporated.

  15. Unruptured aneurysms with cranial nerve symptoms: efficacy of endosaccular guglielmi detachable coil treatment

    International Nuclear Information System (INIS)

    Kim, Dong Joon; Kim, Dong Ik; Lee, Seung Koo; Kim, Si Yeon

    2003-01-01

    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

  16. Spinal Cord Injury due to Tumour or Metastasis in Aragón, Northeastern Spain (1991–2008: Incidence, Time Trends, and Neurological Function

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    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.

  17. Intramedullary Recurrence of a Thoracic Meningioma-Presentation of an Unusual Case and Review of the Literature.

    Science.gov (United States)

    Piazza, Matthew A; Ramayya, Ashwin G; Geiger, Geoffrey A; Alonso-Basanta, Michelle; Nasrallah, MacLean P; Welch, William C; Ozturk, Ali K

    2016-08-01

    Spinal meningiomas are typically extra-axial, slow-growing, benign tumors that arise from the arachnoid cap cells. Intramedullary spinal meningiomas are exceedingly rare with few cases reported in the literature. A 64-year-old man with a history of grade I thoracic meningioma at the T4 level resected initially in 1989 and who required reoperation in 2013 for intradural, extramedullary recurrence of tumor presented again in 2015 with gait difficulty. Magnetic resonance imaging revealed a soft tissue mass at the T3 to T4 levels on the left side of the canal that was mildly enhancing on T1 contrasted sequences. The patient was taken to the operating room, where a purely intramedullary recurrence was discovered without extramedullary extension or a dural-based attachment. The intramedullary tumor was completely resected, and postoperatively the patient recovered well and was at his neurologic baseline. The patient ultimately underwent proton beam radiotherapy because this tumor, although benign, had recurred twice. Intramedullary spinal meningiomas, particularly intramedullary low-grade recurrence of a previously extramedullary tumor, are rare phenomena. Although the pathogenic mechanisms are not well understood, intramedullary recurrence as described in this patient may reflect extrinsic factors related to prior surgical resections in addition to histologic progression. When operating on recurrent extramedullary lesions, aggressive arachnoid dissection may predispose patients to unusual patterns of recurrence. Copyright © 2016 Elsevier Inc. All rights reserved.

  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. Science.gov (United States)

    Zanoletti, E.; Borsetto, D.; Opocher, G.; Mazzoni, A.; Martini, A.

    2017-01-01

    SUMMARY 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. PMID:29165437

  20. The use of an intraarticular catheter on fast-track primary knee arthroplasty, is it a step forward?

    Science.gov (United States)

    Cáceres-Sánchez, L; García-Benítez, J B; Coronado-Hijón, V; Montero-Pariente, M

    This study compares the efficacy in post-operative pain control of the intraarticular catheter compared to the epidural catheter after primary total knee arthroplasty. Randomised clinical trial consisting of two groups of patients. A control group with intradural anaesthesia and an intraoperative epidural catheter (ropivacaine) and an intervention group using the same anaesthetic technique and an intraarticular catheter with an elastomeric pump (ropivacaine+dexketoprofen). Data such as demographic, anaesthetic and surgical variables, pain intensity according to Verbal Rating Scale, opioid use and complications, joint balance, onset of walking and hospital stay were recorded. A lower incidence and severity on Verbal Rating Scale and a better control of postoperative pain (p.001). Finally, the hospital stay decreased significantly, with 33.3% of intervention group patients discharged within the first 48h compared to none of the control group. The use of the intraarticular catheter as postoperative analgesia is a useful and safe alternative. It reduces the possibility of side effects. It helps in early improvement of joint balance, onset of walking and control of pain. All of which increase patient satisfaction and result in a shorter period of hospitalisation. Copyright © 2017 SECOT. Publicado por Elsevier España, S.L.U. All rights reserved.

  1. Transmastoid approach to temporal bone cerebrospinal fluid leaks.

    Science.gov (United States)

    Oliaei, Sepehr; Mahboubi, Hossein; Djalilian, Hamid R

    2012-01-01

    The aim of the study was to evaluate various presentations and treatment options for spontaneous cerebrospinal fluid (CSF) leakage originating in the temporal bone. Clinical data and imaging results for 18 ears (15 patients) presenting with spontaneous CSF leakage originating in the temporal bone were reviewed. Average follow-up period was 13.5 months. The main outcome measure was presence of persistent CSF leak postoperatively. A standard postauricular mastoidectomy was performed. Fifteen patients diagnosed with spontaneous CSF leakage over an 8-year period including 3 treated for bilateral disease were included in the study. The age ranged between 33 and 83 years. Presenting symptoms included serous otitis media (44%), persistent otorrhea after tympanostomy tube placement (28%), and meningitis (28%). Preoperative diagnosis was made using imaging studies and was substantiated by observation of CSF leakage and dural herniation intraoperatively. Treatment was eustachian tube plugging (5%), mastoidectomy with fat obliteration (61%), middle fossa approach with extradural (17%), intradural repair (5%), or combined middle fossa and transmastoid (TM) approach (11%). Successful treatment was obtained in 17 of the 18 cases. The last 9 patients in the series underwent TM approach alone for repair with no treatment failures. Repair of defects in tegmen mastoideum and posterior fossa can be successfully achieved on an outpatient basis without regard to size and multitude of defects via TM approach. This approach obviates the need for a craniotomy or lumbar drain. Copyright © 2012 Elsevier Inc. All rights reserved.

  2. A New Classification for Pathologies of Spinal Meninges, Part 1: Dural Cysts, Dissections, and Ectasias.

    Science.gov (United States)

    Klekamp, Jörg

    2017-07-01

    The clinical significance of pathologies of the spinal dura is often unclear and their management controversial. To classify spinal dural pathologies analogous to vascular aneurysms, present their symptoms and surgical results. Among 1519 patients with spinal space-occupying lesions, 66 patients demonstrated dural pathologies. Neuroradiological and surgical features were reviewed and clinical data analyzed. Saccular dural diverticula (type I, n = 28) caused by defects of both dural layers, dissections between dural layers (type II, n = 29) due to defects of the inner layer, and dural ectasias (type III, n = 9) related to structural changes of the dura were distinguished. For all types, symptoms consisted of local pain followed by signs of radiculopathy or myelopathy, while one patient with dural ectasia presented a low-pressure syndrome and 10 patients with dural dissections additional spinal cord herniation. Type I and type II pathologies required occlusion of their dural defects via extradural (type I) or intradural (type II) approaches. For type III pathologies of the dural sac no surgery was recommended. Favorable results were obtained in all 14 patients with type I and 13 of 15 patients with type II pathologies undergoing surgery. The majority of dural pathologies involving root sleeves remain asymptomatic, while those of the dural sac commonly lead to pain and neurological symptoms. Type I and type II pathologies were treated with good long-term results occluding their dural defects, while ectasias of the dural sac (type III) were managed conservatively. Copyright © 2017 by the Congress of Neurological Surgeons

  3. Spinal meningeal melanocytoma in a 5-year-old child: a case report and review of literature.

    Science.gov (United States)

    Salah El-Din, Ahmed M; Aboul-Ela, Hashem M; Alsawy, Mohamed F; Koheil, Ahmed; Ashry, Ahmed H

    2018-01-01

    Meningeal melanocytoma is considered a rare lesion arising from leptomeningeal melanocytes. Nearly two thirds of meningeal melanocytomas were reported in the intracranial compartment and the remaining one third in the spine. Spinal melanocytomas can be extradural or intradural, with extradural variant being more common, and the majority of cases have been single reports. A 5-year-old male presented with a 4-month history of non-radiating low back pain persistent at rest, with otherwise non-remarkable medical history. The patient was neurologically intact with no deficits. Preoperatively, routine laboratory investigations were non-remarkable. MRI imaging was done and showed a lesion at the level of T11 to L4, hyperintense on T1 and hypointense on T2 with homogenous contrast enhancement. Intraoperatively, the lesion was hemorrhagic, brownish, and rubbery in consistency attached to the ventral dura. Microscopic picture revealed dense cytoplasmic brown melanin pigments, with no significant mitoses or nuclear atypia. What is unique about our case is the age of the patient (5 years). To the best of our knowledge, after reviewing the literature, this is the youngest case to be reported. SMM is an extremely rare tumor with a benign course. Complete surgical excision should be attempted. Age of presentation may be as young as in our case and the diagnosis of such a tumor should never be excluded in this early age group with persistent low back ache.

  4. Spinal dermoid cyst. Characteristic CT findings after metrizamide myelography

    Energy Technology Data Exchange (ETDEWEB)

    Miyamoto, Yoshihisa; Makita, Yasumasa; Nabeshima, Sachio; Tei, Taikyoku; Keyaki, Atsushi; Takahashi, Jun; Kawamura, Junichiro

    1987-10-01

    A 25-year-old male complained of intermittent, sharp pains about the left eye and in the left side of the chest. Neurological examination revealed paresthesia and impaired perception of touch and pin-pricks in the dermatomes of Th8 and Th9 on the left side. In all four extremities, the muscle stretch reflexes were equal and slightly hyperactive, without weakness or sensory deficits. Metrizamide myelography showed defective filling at the level between the upper 8th and 9th thoracic vertebrae. The lesion was also demonstrated by computed tomography (CT) scan performed 1 hour later, appearing as an oval, radiolucent mass in the left dorsal spinal canal, which compressed the spinal cord forward and toward the right. Serial sections of the spinal canal revealed the lesion to be partly filled with contrast medium. Repeat CT scan 24 hours after metrizamide myelography showed more contrast medium in the periphery of the lesion, giving it a doughnut-shaped appearance. At surgery a smooth-surfaced cyst containing sebum and white hair was totally removed from the intradural extramedullary space. The histological diagnosis was dermoid cyst. There have been a few reported cases of intracranial epidermoid cyst in which filling of the cyst was suggested on metrizamide CT myelography. These findings may complicate the differential diagnosis of arachnoid cyst and dermoid or epidermoid cyst when only CT is used.

  5. 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.)

  6. 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.

  7. Spinal meningiomas in dogs: 13 cases (1972-1987)

    Energy Technology Data Exchange (ETDEWEB)

    Fingeroth, J. M.; Prata, R. G.; Patnaik, A. K.

    1987-09-15

    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.

  8. 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.

  9. Endoscopic endonasal skull base surgery: advantages, limitations, and our techniques to overcome cerebrospinal fluid leakage: technical note.

    Science.gov (United States)

    Ishii, Yudo; Tahara, Shigeyuki; Teramoto, Akira; Morita, Akio

    2014-01-01

    In recent years, resections of midline skull base tumors have been conducted using endoscopic endonasal skull base (EESB) approaches. Nevertheless, many surgeons reported that cerebrospinal fluid (CSF) leakage is still a major complication of these approaches. Here, we report the results of our 42 EESB surgeries and discuss the advantages and limits of this approach for resecting various types of tumors, and also report our technique to overcome CSF leakage. All 42 cases involved midline skull base tumors resected using the EESB technique. Dural incisions were closed using nasoseptal flaps and fascia patch inlay sutures. Total removal of the tumor was accomplished in seven pituitary adenomas (33.3%), five craniopharyngiomas (62.5%), five tuberculum sellae meningiomas (83.3%), three clival chordomas (100%), and one suprasellar ependymoma. Residual regions included the cavernous sinus, the outside of the intracranial part of the internal carotid artery, the lower lateral part of the posterior clivus, and the posterior pituitary stalk. Overall incidence of CSF leakage was 7.1%. Even though the versatility of the approach is limited, EESB surgery has many advantages compared to the transcranial approach for managing mid-line skull base lesions. To avoid CSF leakage, surgeons should have skills and techniques for complete closure, including use of the nasoseptal flap and fascia patch inlay techniques.

  10. CT and MR imaging findings of sphenoidal masses

    Energy Technology Data Exchange (ETDEWEB)

    Takahashi, Shoki; Higano, Shuichi (Tohoku Univ., Sendai (Japan). School of Medicine); Ishii, Kiyoshi (and others)

    1994-07-01

    CT and MR imaging findings of 57 sphenoidal masses were retrospectively reviewed to assess the possibility of differential diagnosis between them. Various kinds of masses such as pituitary adenoma, epipharyngeal cancer, mucocele, chordoma, chondroma, chondrosarcoma, distant metastasis, multiple myeloma, fibrous dysplasia, craniopharyngioma, hemangiopericytoma, giant cell tumor, primary sphenoidal cancer, malignant melanoma, leukemia, histiocytosis X, and giant cell tumor were included in this series. CT scanning was performed in all cases using a spin-echo pulse sequence. The relative density of the masses, bony changes and calcification were evaluated on CT, and on MR images, signal intensity of the masses relative to the normal gray matter, contrast enhancement and extension/contour were evaluated. Although no single feature appeared to be specific to the masses, detection of calcification on CT, identification of the normal pituitary gland as deformed or displaced on T1-weighted images, signal intensity on T2-weighted images, and extension of the masses seemed to be useful and should be examined in terms of their ability to assist in differential diagnosis. Finally, accommodative classification of sphenoidal masses primarily based on presumed origin or mode of extension was attempted. (author).

  11. 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.

  12. Is proton beam therapy the future of radiotherapy? Part I: Clinical aspects; La protontherapie: avenir de la radiotherapie? Premiere partie: aspects cliniques

    Energy Technology Data Exchange (ETDEWEB)

    Bouyon-Monteau, A.; Habrand, J.L.; Datchary, J.; Alapetite, C.; Bolle, S.; Dendale, R.; Feuvret, L.; Helfre, S.; Calugaru, V. [Centre de protontherapie d' Orsay, institut Curie, campus universitaire, 91 - Orsay (France); Bouyon-Monteau, A.; Alapetite, C.; Bolle, S.; Dendale, R.; Helfre, S.; Calugaru, V.; Cosset, J.M.; Bey, P. [Departement d' oncologie-radiotherapie, institut Curie, 75 - Paris (France); Habrand, J.L.; Datchary, J. [Departement d' oncologie-radiotherapie, institut de cancerologie Gustave-Roussy, 94 - Villejuif (France); Feuvret, L. [Departement d' oncologie-radiotherapie, hopital Pitie-Salpetriere, 75 - Paris (France)

    2010-12-15

    Proton beam therapy uses positively charged particles, protons, whose physical properties improve dose-distribution (Bragg peak characterized by a sharp distal and lateral penumbra) compared with conventional photon-based radiation therapy (X-ray). These ballistic advantages apply to the treatment of deep-sited tumours located close to critical structures and requiring high-dose levels. [60-250 MeV] proton-beam therapy is now widely accepted as the 'gold standard' in specific indications in adults - ocular melanoma, chordoma and chondrosarcoma of the base of skull - and is regarded as a highly promising treatment modality in the treatment of paediatric malignancies (brain tumours, sarcomas..). This includes the relative sparing of surrounding normal organs from low and mid-doses that can cause deleterious side-effects such as radiation-induced secondary malignancies. Other clinical studies are currently testing proton beam in dose-escalation evaluations, in prostate, lung, hepatocellular cancers, etc. Clinical validation of these new indications appears necessary. To date, over 60, 000 patients worldwide have received part or all of their radiation therapy program by proton beams, in approximately 30 treatment facilities. (authors)

  13. Percutaneous gastrostomy -a report of twenty-seven cases-

    International Nuclear Information System (INIS)

    Kim, Tae Ho; Lee, Ho Suk; Kim, Yong Joo; Kim, Tae Hun; Suh, Kyung Jin; Kang, Duk Sik

    1991-01-01

    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

  14. Study on blood supply of lung metastasis with trans-pulmonary arterial lipiodol infusion

    International Nuclear Information System (INIS)

    Zhou Jianqin; Dong Weihua; Dong Weihua; Ouyang Chang; Chang Heng; Xiao Xiangsheng

    2008-01-01

    Objective: To evaluate the blood supply of pulmonary metastases using small volume of lipiodol through pulmonary arterial infusion. Methods: 10 cases of lung metastasis were enroled including the primary tumors of liver cancer (n=5), renal carcinoma (n=3), chordoma (n=1) and malignant neurofibroma (n=1). Plain CT scan was performed to exclude calcification or ossification within metastasis and then pulmonary arterial DSA was undertaken to evaluate tumor vessels or staining. After pulmonary arteriovenous fistula or other anomalous circulation was excluded by lobar arterial DSA, small volume of lipiodol was infused under fluoroscopy (0.5-1.5 ml for each lobar artery, total volume less than 3.0 ml). CT scan was immediately performed. Blood supply of the pulmonary metastases was assessed according to the accumulation of lipiodol on CT scans. Results: No cases but one experienced cough, expectoration, suffocating or dyspnea. No complication of cerebral or visceral embolism occurred. Totally 27 nodules were studied including 6 nodules with cloudy lipiodol accumulation and 6 nodules with tiny granules of lipiodol accumulation. No enlarged tumor vessel or tumor stain was observed within all 27 nodules on pulmonary arterial DSA. Conclusions: Pulmonary artery supplys only parts of pulmonary metastases, especially those sited at the peripheral region of the lung. Infusion of small volume of lipiodol through pulmonary artery is safe, and the increased density of lung field could return normal after several days. (authors)

  15. Morphological studies in the diagnosis of primary and secondary bone tumors

    Directory of Open Access Journals (Sweden)

    Matveeva O.V.

    2016-12-01

    Full Text Available The aim: to show the possibility of morphological studies in the diagnosis of primary and secondary tumors of bones. Material and Methods. 105 (72% patients with primary bone tumors aged from 15 to 66 years and 42 (28% patients with metastatic bone lesions aged from 42 to 70 years were examined and treated for the period from 2008 till 2015. Material for morphological studies was prepared using an open biopsy tissue slices and a scraping resected tumor during surgery. Soft-tissue component is subjected to cytology. The material for histological study included changes in bone and soft tissue. Results. Giant cell tumor was verified in 45% of cases by histological examination. Multiple myeloma was diagnosed in 15% of patients. Osteogenic sarcoma was diagnosed in 14% of cases. Ewing's sarcoma was diagnosed in 3%, 2% of cases were matched by diagnosed chordoma. According to the data received, cancer metastasis of kidney and lung is mostly diagnosed in men from the group of patients with secondary bone defeat. Metastasis of cancer of the breast in women was predominated. Conclusion. The morphological (histological, cytological study plays an important role in the diagnosis of bone tumors. The coincidence of the cytological and histological diagnoses was 97%.

  16. Polymer Nanoparticle-Based Chemotherapy for Spinal Malignancies

    Directory of Open Access Journals (Sweden)

    Hongyun Ma

    2016-01-01

    Full Text Available Malignant spinal tumors, categorized into primary and metastatic ones, are one of the most serious diseases due to their high morbidity and mortality rates. Common primary spinal tumors include chordoma, chondrosarcoma, osteosarcoma, Ewing’s sarcoma, and multiple myeloma. Spinal malignancies are not only locally invasive and destructive to adjacent structures, such as bone, neural, and vascular structures, but also disruptive to distant organs (e.g., lung. Current treatments for spinal malignancies, including wide resection, radiotherapy, and chemotherapy, have made significant progress like improving patients’ quality of life. Among them, chemotherapy plays an important role, but its potential for clinical application is limited by severe side effects and drug resistance. To ameliorate the current situation, various polymer nanoparticles have been developed as promising excipients to facilitate the effective treatment of spinal malignancies by utilizing their potent advantages, for example, targeting, stimuli response, and synergetic effect. This review overviews the development of polymer nanoparticles for antineoplastic delivery in the treatment of spinal malignancies and discusses future prospects of polymer nanoparticle-based treatment methods.

  17. Evaluation of the MRI with Gd-DTPA enhancement in the diagnosis of brain and spinal tumors

    Energy Technology Data Exchange (ETDEWEB)

    Fukui, Keiji; Sadamoto, Kazuhiko; Miki, Hitoshi; Sakaki, Saburo; Matsuoka, Kenzo.

    1988-02-01

    Magnetic resonance (MR) imaging was performed using saturation recovery (SR), inversion recovery (IR) and spin echo (SE) pulse sequences before and after the injection of 0.1 mmol of gadolinium diethylenetriamine pentaacetic acid dimeglumine (Gd-DTPA) per kilogram of body weight. Forty-two patients with gliomas (8), meningiomas (12), neurinomas (5), adenoma (1), craniopharyngioma (1), chordoma (1), malignant lymphomas (2), metastatic brain tumors (2), spinal tumors (4), and other tumors (6) were studied. Contrast enhancement was shown in 35 of the 38 patients with brain tumors and in all those with spinal tumors on T/sub 1/-weighted images. The T/sub 1/ relaxation time was decreased in all of these 39 tumors, while the T/sub 2/ relaxation time showed no definite tendency. No marked side effects were observed following the administration of Gd-DTPA. By using Gd-DTPA enhancement, it was possible to differentiate the tumor from the peritumoral edema on MRI, although it was difficult to do so on precontrast MRI. Among the various pulse sequences, the SR sequence provides the best diagnostic value with Gd-DTPA enhancement in the shortest examination time for screening brain and spinal tumors.

  18. 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.

  19. Cytokeratin positivity in myxopapillary ependymoma – a potential diagnostic pitfall

    Directory of Open Access Journals (Sweden)

    Sur Monalisa

    2008-10-01

    Full Text Available Abstract Background Myxopapillary ependymomas (MPE occur in the filum terminale of the spinal cord, but also present in extra-spinal locations such as subcutaneous tissue and brain. They are slow growing grade I gliomas. Areas of solid growth pattern with aggregates of cells with "epithelioid morphology" seen in MPE can mimic metastatic carcinoma. The presence of occasional cells with clear cytoplasm and morphology can resemble Chordoma. Diagnosis can be missed due to these morphological similarities, which could affect patient management and hence, long term survival. Case presentation We describe two cases of MPE with cytokeratin (AE1 AE3, CAM 5.2, Cytokeratin 7 and cytokeratin 20 expression. Conclusion MPE can be positive for Cytokeratins (CAM 5.2, AE1 AE3, CK7 and focally for EMA, which could be misdiagnosed as metastatic carcinoma. In cases demonstrating epithelioid and clear cell morphology, the diagnosis of MPE should be made in conjunction with histology, proper immunohistochemical profile which includes co-expression of GFAP, S-100 protein and epithelial markers, radiologic findings and site. It is important to be aware of the cytokeratin profile in MPE to avoid erroneous diagnosis with other tumour entities.

  20. Cytomorphologic features of myxopapillary ependymoma: a review of 13 cases.

    Science.gov (United States)

    Takei, Hidehiro; Kosarac, Ognjen; Powell, Suzanne Z

    2009-01-01

    To describe the cytologic features of myxopapillary ependymoma (MPE) on intraoperative smears, to analyze cytomorphologic parameters that may help in reaching the diagnosis and to discuss differential diagnosis. Touch imprint smears of 13 MPE cases were reviewed and graded semiquantitatively for 14 cytomorphologic parameters; cellularity, myxoid background, isolated/dispersed tumor cells, "hyaline globules (HGs)," fibrillary cytoplasmic processes, papillary structures, perivascular pseudorosettes, epithelioid tumor cells (ETCs), intracytoplasmic mucin, intranuclear inclusions, nuclear grooves, mitosis, cytologic atypia and hemosiderin-laden macrophages. Cytologic examination revealed variably cellular specimens composed of isolated and loosely aggregated tumor cells with round to oval or occasionally spindle-shaped nuclei; evenly distributed, finely granular chromatin; and fibrillary processes admixed with occasional ETCs. Most of the cases showed prominent fibrillary processes and occasional ETCs with at least a focal myxoid background. HGs and hemosiderin-laden macrophages were often seen. Papillary structure, a histologic hallmark of MPE, was rarely observed. Dual glial and epithelioid properties of tumor cells, well-known features of "regular" ependymomas, and a distinctive myxoid background with HGs strongly support a diagnosis of MPE and are of great help in excluding other mimics (e.g., other variants of ependymoma, metastatic mucinous adenocarcinoma, metastatic adenoid cystic carcinoma and chordoma).

  1. 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.

  2. The role of stereotactic radiosurgery in the treatment of malignant skull base tumors

    International Nuclear Information System (INIS)

    Miller, Robert C.; Foote, Robert L.; Coffey, Robert J.; Gorman, Deborah A.; Earle, John D.; Schomberg, Paula J.; Kline, Robert W.

    1997-01-01

    Purpose: To determine the efficacy and toxicity of stereotactic radiosurgery in the treatment of malignant skull base tumors. Methods and Materials: Thirty-two patients with 35 newly diagnosed or recurrent malignant skull base tumors ≤33.5 cm 3 were treated using the Leksell Gamma unit. Tumor histologies included: adenoid cystic carcinoma, basal cell carcinoma, chondrosarcoma, chordoma, nasopharyngeal carcinoma, osteogenic sarcoma, and squamous cell carcinoma. Results: After a median follow-up of 2.3 years, 83% ± 15% (±95% confidence interval) of patients experienced a symptomatic response to treatment. Local control at the skull base was 95 ± 9% at 2 years and 78 ± 23% at 3 years. Local-regional control above the clavicles was 75 ± 15% at 1 year and 51 ± 20% at 2 years. Overall and cause specific survival were identical, 82 ± 13% at 1 year, 76 ± 14% at 2 years, and 72 ± 16% at 3 years. One patient developed a radiation-induced optic neuropathy 12 months after radiosurgery. Conclusion: Stereotactic radiosurgery using the Leksell Gamma Unit can provide durable tumor control and symptomatic relief with acceptable toxicity in the majority of patients with malignant tumors 4 cm or less in size involving the skull base. Further evaluation of more patients with longer follow-up is warranted

  3. 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.

  4. 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

  5. Role of the ECM in notochord formation, function and disease.

    Science.gov (United States)

    Trapani, Valeria; Bonaldo, Paolo; Corallo, Diana

    2017-10-01

    The notochord is a midline structure common to all chordate animals; it provides mechanical and signaling cues for the developing embryo. In vertebrates, the notochord plays key functions during embryogenesis, being a source of developmental signals that pattern the surrounding tissues. It is composed of a core of vacuolated cells surrounded by an epithelial-like sheath of cells that secrete a thick peri-notochordal basement membrane made of different extracellular matrix (ECM) proteins. The correct deposition and organization of the ECM is essential for proper notochord morphogenesis and function. Work carried out in the past two decades has allowed researchers to dissect the contribution of different ECM components to this embryonic tissue. Here, we will provide an overview of these genetic and mechanistic studies. In particular, we highlight the specific functions of distinct matrix molecules in regulating notochord development and notochord-derived signals. Moreover, we also discuss the involvement of ECM synthesis and its remodeling in the pathogenesis of chordoma, a malignant bone cancer that originates from remnants of notochord remaining after embryogenesis. © 2017. Published by The Company of Biologists Ltd.

  6. Neurophysiological Identification of Cranial Nerves During Endoscopic Endonasal Surgery of Skull Base Tumors: Pilot Study Technical Report.

    Science.gov (United States)

    Shkarubo, Alexey Nikolaevich; Chernov, Ilia Valerievich; Ogurtsova, Anna Anatolievna; Moshchev, Dmitry Aleksandrovich; Lubnin, Andrew Jurievich; Andreev, Dmitry Nicolaevich; Koval, Konstantin Vladimirovich

    2017-02-01

    Intraoperative identification of cranial nerves is crucial for safe surgery of skull base tumors. Currently, only a small number of published papers describe the technique of trigger electromyography (t-EMG) in endoscopic endonasal removal of such tumors. To assess the effectiveness of t-EMG in preventing intraoperative cranial nerve damage in endoscopic endonasal surgery of skull base tumors. Nine patients were operated on using the endoscopic endonasal approach within a 1-year period. The tumors included large skull base chordomas and trigeminal neurinomas localized in the cavernous sinus. During the surgical process, cranial nerve identification was carried out using monopolar and bipolar t-EMG methods. Assessment of cranial nerve functional activity was conducted both before and after tumor removal. We mapped 17 nerves in 9 patients. Third, fifth, and sixth cranial nerves were identified intraoperatively. There were no cases of postoperative functional impairment of the mapped cranial nerves. In one case we were unable to get an intraoperative response from the fourth cranial nerve and observed its postoperative transient plegia (the function was normal before surgery). t-EMG allows surgeons to control the safety of cranial nerves both during and after skull base tumor removal. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. TRANSORAL REMOVAL OF SKULL BASE AND C1-C2 VERTEBRAL BODY TUMOURS AND NONTUMOROUS PATHOLOGY IN THE CRANIOCERVICAL JUNCTION ACCOMPANIED BY CRANIOVERTEBRAL INSTABILITY

    Directory of Open Access Journals (Sweden)

    A. N. Shkarubo

    2010-01-01

    Full Text Available 27 patients aged 2,5-61 years with skull base and C1-C2 vertebral body tumours and nontumorous pathology in the craniocervical junction underwent surgery. All patients revealed craniovertebral instability. To perform OSD we used autobone and metallic wire in 1 case, "Ventrofix" - 2; "CCD" - 9, "Vertex" - 15. In 26 cases OSD was followed by transoral tumor removal; in 1 - removal of the skull base chordoma spreading into C1-C2 segments was followed by OSD. In our practice we used original patent instruments, devices and surgical techniques. After the tumor has been removed, the skull defect hermetic closure and plasty were performed using the original patent technique for preventing postoperative CSF leakage as well as different glue compositions. This technique proved to shorten hospitalization period and reduce treatment costs as well as launch an early rehabilitation programme - on the 3d-4th day after operation. Use of new technologies in surgical treatment of skull base tumors invading upper cervical spinal segments accompanied by craniovertebral instability allowed to improve surgical outcome and start up early rehabilitation.

  8. 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

  9. Profile of European proton and carbon ion therapy centers assessed by the EORTC facility questionnaire.

    Science.gov (United States)

    Weber, Damien C; Abrunhosa-Branquinho, André; Bolsi, Alessandra; Kacperek, Andrzej; Dendale, Rémi; Geismar, Dirk; Bachtiary, Barbara; Hall, Annika; Heufelder, Jens; Herfarth, Klaus; Debus, Jürgen; Amichetti, Maurizio; Krause, Mechthild; Orecchia, Roberto; Vondracek, Vladimir; Thariat, Juliette; Kajdrowicz, Tomasz; Nilsson, Kristina; Grau, Cai

    2017-08-01

    We performed a survey using the modified EORTC Facility questionnaire (pFQ) to evaluate the human, technical and organizational resources of particle centers in Europe. The modified pFQ consisted of 235 questions distributed in 11 sections accessible on line on an EORTC server. Fifteen centers from 8 countries completed the pFQ between May 2015 and December 2015. The average number of patients treated per year and per particle center was 221 (range, 40-557). The majority (66.7%) of centers had pencil beam or raster scanning capability. Four (27%) centers were dedicated to eye treatment only. An increase in the patients-health professional FTE ratio was observed for eye tumor only centers when compared to other centers. All centers treated routinely chordomas/chondrosarcomas, brain tumors and sarcomas but rarely breast cancer. The majority of centers treated pediatric cases with particles. Only a minority of the queried institutions treated non-static targets. As the number of particle centers coming online will increase, the experience with this treatment modality will rise in Europe. Children can currently be treated in these facilities in a majority of cases. The majority of these centers provide state of the art particle beam therapy. Copyright © 2017 Elsevier B.V. All rights reserved.

  10. 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

  11. Recurrent spinal primitive neuroectodermal tumor with brain and bone metastases: A case report.

    Science.gov (United States)

    Chen, Frank; Chiou, Shyh-Shin; Lin, Sheng-Fung; Lieu, Ann-Shung; Chen, Yi-Ting; Huang, Chih-Jen

    2017-11-01

    Primary spinal primitive neuroectodermal tumor (PNET) is relatively rare in all age groups, and the prognosis in most cases of spinal PNETs appears to be poor, with a median patient survival of 1 to 2 years. We present a case with recurrent spinal PNET with brain and bone metastases that was successfully treated by multimodality treatment. A 14-year-old teenage girl had suffered from progressive left upper back pain with bilateral lower legs weakness and numbness for 1 year. After treatment, left neck mass was noted 3 years later. Initially, magnetic resonance imaging (MRI) showed neurogenic tumor involving intradural extramedullary space of T5-T10. Pathology report showed PNET (World Health Organization grade IV) featuring lobules of neoplastic cells with round regular nuclei, high nucleus-to-cytoplasm ratio, and fibrillary cytoplasm. At the time of tumor recurrence, chest MRI then showed recurrent tumor at T2-T3 level of the epidural space with right neural foramina invasion. Brain MRI showed extensive bilateral calvarial metastases and leptomeningeal metastases in the right frontoparietal regions. Bone scan showed multiple bone metastases. T-spine tumor removal and adjuvant radiotherapy (RT) to T-spine tumor bed were performed in the initial treatment. After clinical tumor recurrence, tumor removal was done again. She then received chemotherapy followed by whole brain irradiation with hippocampal sparing with 35 gray in 20 fractions. After treatment, follow-up images showed that the disease was under control. There was no neurological sequela. She has survived more than 7 years from diagnosis and more than 4 years from recurrence to date. Multimodality treatments including operation, RT, and chemotherapy should be considered in the initial treatment planning, and salvage chemotherapy was useful in this case.

  12. Early harvesting of the vascularized pedicled nasoseptal flap during endoscopic skull base surgery.

    Science.gov (United States)

    Eloy, Jean Anderson; Patel, Amit A; Shukla, Pratik A; Choudhry, Osamah J; Liu, James K

    2013-01-01

    The vascularized pedicled nasoseptal flap (PNSF) represents a successful option for reconstruction of large skull base defects after expanded endoscopic endonasal approaches (EEA). This vascularized flap can be harvested early or late in the operation depending on the anticipation of high-flow CSF leaks. Each harvesting technique (early vs. late) is associated with different advantages and disadvantages. In this study, we evaluate our experience with early harvesting of the PNSF for repair of large skull base defects after EEA. A retrospective review was performed at a tertiary care medical center on patients who underwent early PNSF harvesting during reconstruction of intraoperative high-flow CSF leaks after EEA between December 2008 and March 2012. Demographic data, repair materials, surgical approach, and incidence of PNSF usage were collected. Eighty-seven patients meeting the inclusion criteria were identified. In 86 procedures (98.9%), the PNSF harvested at the beginning of the operation was used. In 1 case (1.1%), the PNSF was not used because a high-flow intraoperative CSF leak was not encountered. This patient had recurrence of intradural disease 8months later, and the previously elevated PNSF was subsequent used after tumor resection. Based on our data, a high-flow CSF leak and need for a PNSF can be accurately anticipated in patients undergoing EEA for skull base lesions. Because of the advantages of early harvesting of the PNSF and the high preoperative predictive value of CSF leak anticipations, this technique represents a feasible harvesting practice for EEA surgeries. Copyright © 2013 Elsevier Inc. All rights reserved.

  13. Radiologic evaluation of spinal epidural mass

    International Nuclear Information System (INIS)

    Lee, Ho Kyu; Lee, Moon Kyu; Chang, Kee Hyun

    1987-01-01

    It is often difficult to differentiate each pathologic entity among various spinal epidural masses on the radiologic basis. We retrospectively analysed radiologic findings of 67 cases of pathologically proven spinal epidural mass to find out any specific findings of each epidural mass. The results are as follows : 1. Of 67 cases, metastasis (16 cases), epidural abscess (15 cases), neurogenic tumor (16 cases) and meningioma (8 cases) are most common. The others consist of epidural angioma (3 cases), vertebral hemangioma (2 cases), chondrosarcoma (2 cases), fibrosarcoma (1 case), eosinophilic granuloma (1 case), lymphoma (1 case), lipoma (1 case) and lymphoid hyperplasia (1 case). 2. Destruction of the adjacent bone was commonly associated with the epidural mass in metastasis (75%) and epidural abscess (67%). It was also noted in 2 cases of chondrosarcoma, 1 fibrosarcoma and 1 eosinophilic granuloma. 3. Pressure erosion of adjacent bone was demonstrated in neurogenic tumor (75%) and meningioma (25%). 4. Narrowing of intervertebral space was seen in only 27% of epidural abscess. 5. The paraspinal tumor was associated in 67% of metastasis, 80% of epidural abscess, 75% of neurogenic tumor, 33% of meningioma. It was also seen in 2 cases of chondrosarcoma, 1 fibrosarcoma and 1 eosinophilic granuloma. 6. The intradural tumor was associated in 50% of neurogenic tumor and 67% of meningioma. 7. On axial CT image, most of the epidural mass shows eccentric location with displacing dural sac to the opposite side. The diseases that occasionally show encircling location are metastasis, epidural abscess, vertebral hemangioma, chondrosarcoma, eosinophilic granuloma, and lymphoma. Neurogenic tumor only shows multicentric location. 8. The disease extent more than height of one vertebral body was seen in 80% of epidural abscess, 58% of neurogenic tumor, 100% of epidural angioma. It was also seen in 2 cases of chondrosarcoma, 1 fibrosarcoma, 1 eosinophilic granuloma, 1 lymphoma, 1

  14. Brown-Séquard syndrome following intracranial subarachnoid hemorrhage-induced spinal arachnoid cyst

    Directory of Open Access Journals (Sweden)

    Yew-Weng Fong

    2017-12-01

    Full Text Available Objective: Arachnoid cyst associated with spinal arachnoiditis following subarachnoid hemorrhage (SAH is a rare complication. Correctly diagnosing this condition and providing an effective treatment are extremely important. Case report: A 60-year-old women had a history of SAH due to an intradural vertebral artery dissecting aneurysm post-coil embolization, without neurologic deficits on discharge from our institution. She experienced left hemiparesis with muscle power graded at 4/5 eight months later due to a right middle cerebral artery territory infarct. Nine months after stroke rehabilitation, there was a progression of left-side weakness and right-side numbness down below the nipple level. On neurological examination, there were right sensory deficits below T6, and left hemiparesis with muscle power of the left upper extremity graded at 4/5 and that of the left lower extremity graded at 3/5. Deep tendon reflex was symmetrically increased at the bilateral lower extremities. Spinal magnetic resonance imaging (MRI revealed a cervicothoracic multiseptated arachnoid cyst with a major compression effect at T6. Eight months after T5–6 hemilaminectomy and fenestration of the cyst wall, the neurologic deficits had not improved. The patient then underwent a cysto-peritoneal shunt. A follow-up MRI documented a complete resolution of the arachnoid cyst. The patient's Brown-Séquard syndrome showed clinical improvement. Conclusion: Prompt investigation revealed a spinal arachnoid cyst in a patient with a history of intracranial SAH presenting with unexplained progressive Brown-Séquard syndrome. For cases of multiseptated and long arachnoid cyst, as in our patient, a shunting procedure is a better choice than fenestration alone.

  15. 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.

  16. Open Approaches to the Anterior Skull Base in Children: Review of the Literature.

    Science.gov (United States)

    Wasserzug, Oshri; DeRowe, Ari; Ringel, Barak; Fishman, Gadi; Fliss, Dan M

    2018-02-01

    Introduction  Skull base lesions in children and adolescents are rare, and comprise only 5.6% of all skull base surgery. Anterior skull base lesions dominate, averaging slightly more than 50% of the cases. Until recently, surgery of the anterior skull base was dominated by open procedures and endoscopic skull base surgery was reserved for benign pathologies. Endoscopic skull base surgery is gradually gaining popularity. In spite of that, open skull base surgery is still considered the "gold standard" for the treatment of anterior skull base lesions, and it is the preferred approach in selected cases. Objective  This article reviews current concepts and open approaches to the anterior skull base in children in the era of endoscopic surgery. Materials and Methods  Comprehensive literature review. Results  Extensive intracranial-intradural invasion, extensive orbital invasion, encasement of the optic nerve or the internal carotid artery, lateral supraorbital dural involvement and involvement of the anterior table of the frontal sinus or lateral portion of the frontal sinus precludes endoscopic surgery, and mandates open skull base surgery. The open approaches which are used most frequently for surgical resection of anterior skull base tumors are the transfacial/transmaxillary, subcranial, and subfrontal approaches. Reconstruction of anterior skull base defects is discussed in a separate article in this supplement. Discussion  Although endoscopic skull base surgery in children is gaining popularity in developed countries, in many cases open surgery is still required. In addition, in developing countries, which accounts for more than 80% of the world's population, limited access to expensive equipment precludes the use of endoscopic surgery. Several open surgical approaches are still employed to resect anterior skull base lesions in the pediatric population. With this large armamentarium of surgical approaches, tailoring the most suitable approach to a

  17. 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 (P<.01) and postoperative morbidity were lower for HoLEP than for adenomectomy. However, the retropubic adenomectomy group had larger initial prostate volumes (P<.001) and shorter surgical times (P<.001). Better surgical performance (P<.001) and a lower incidence of complications were observed in the HoLEP-B group (once the learning curve had been overcome) compared with the HoLEP-A group. In our center, HoLEP was introduced as a valid alternative to open retropubic adenomectomy, with excellent results in terms of morbidity and reduced hospital stay. In terms of the learning curve, we consider that approximately 50 patients (without mentor) is an appropriate cutoff. Local anesthesia is a good choice for the anesthesia technique. Copyright © 2014 AEU. Publicado por Elsevier España, S.L.U. All rights reserved.

  18. 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.

  19. Evaluation of the intraspinal enhancement for medulloblastoma on MR imaging

    International Nuclear Information System (INIS)

    Kim, Hwa Young; Kim, In One; Kim, Woo Sun; Cheon, Jung Eun; Yeon, Kyung Mo

    2004-01-01

    The purpose of this study was to analyze the enhancement pattern of the spinal cord for patients with medulloblastoma, and to correlate the enhancement pattern with cerebrospinal fluid (CSF) tumor seeding. We retrospectively reviewed 84 MR images, including the initial and follow-up studies after chemotherapy or radiation therapy, of 25 patients with medulloblastoma who were aged from 2 to 13 years. We analyzed the spinal leptomeningeal enhancement pattern on the MR images. The leptomeningeal enhancement patterns were categorized into three types: Type, I, fine or discontinuous linear enhancement, and type II, continuous linear or nodular enhancement, and type III, intradural mass formation. We correlated the enhancement pattern on MRI with the results of CSF cytology at the initial and follow-up examinations after treatment. Of total 25 patients, type I enhancement was observed for 14 patients. Twelve patients were negative on the initial CSF cytology and 2 patients were positive. On the follow-up MR studies, 14 patients showed no change or only a slight decrease of enhancement, and all were negative on the follow-up CSF cytology. Type II enhancement patterns were observed in seven patients, and all of them were positive on the initial CSF cytology. On follow-up MR study, one patient revealed an increased enhancement with the positive result on the follow-up CSF cytology, and six patients had decreased enhancement on the follow-up MR studies with negative conversion on the follow-up CSF cytology. Type III enhancement patterns were observed in four patients and all of them were positive on the initial CSF cytology. All four patients with tradural mass formations revealed progression of the lesions on follow-up MR studies, and all of them were positive on the follow-up CSF cytology. Type II and III enhancement patterns always represented CSF seeding and a type I enhancement pattern had a low probability of metastasis

  20. Immunohistochemical features of giant cell ependymoma of the filum terminale with unusual clinical and radiological presentation.

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    Candanedo-Gonzalez, Fernando; Ortiz-Arce, Cindy Sharon; Rosales-Perez, Samuel; Remirez-Castellanos, Ana Lilia; Cordova-Uscanga, Candelaria; Gamboa-Dominguez, Armando

    2017-01-14

    Giant cell ependymoma of the filum terminale is a rare variant, generally manifested as a well-circunscribed intradural mass with an indolent biological behavior. We describe the case of a 48-year-old Mexican female who non-relevant past medical history, that developed a GCE of the filum terminale. Magnetic resonance imaging and computed tomography revealed the presence of an intra-axial tumor extending from L3 to L5 with extra-medullary invasion. Therefore the tumor was considered unresectable and only incisional biopsy was obtained, establishing the tentative diagnosis of a poorly differentiated neoplasia. A second evaluation of the case revealed the presence of numerous non-cohesive pleomorphic giant cells with intranuclear inclusions and broad eosinophilic cytoplasm, alternating with intermediate size cells with round, hyperchromatic nuclei and forming a perivascular pseudo-rosettes pattern. The ependymal phenotype was supported by light microscopy and corroborated by immunohistochemistry analysis. The patient was subsequently treated with radiotherapy 54Gy. She is alive after a 27-month follow-up, with residual disease, difficulty ambulating and pain. GCE of filum terminale may have an atypical clinical and radiological presentation, albeit with invasive characteristics and anaplasia on histologic analysis. However, its biological behavior is indolent and associated to longer survival. Due to the presence of giant cells, the differential diagnosis of other primary neoplasias at that site were considered, including paraganglioma, malignant peripheral nerve sheath tumors as well as metastatic malignant melanoma, adrenal carcinoma, thyroid gland carcinoma and urothelial carcinoma, that may all harbor giant cells.

  1. Disseminated primary diffuse leptomeningeal gliomatosis: a case report with liquid based and conventional smear cytology

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    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.

  2. High lumbar disc herniation coexisting with thoracic meningioma – an issue of differential diagnostic

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    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.

  3. Fuji computed radiography (FCR) for the diagnosis of spinal disorders

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    Kubo, Yoshichika; Waga, Shiro; Kojima, Tadashi

    1987-04-01

    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.

  4. 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

  5. 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)

  6. 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)

  7. 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.

  8. Computed tomography of traumatic extradural hematoma

    International Nuclear Information System (INIS)

    Fukamachi, Akira; Wakao, Tetsuo; Kaneko, Masami; Kunimine, Hideo; Wada, Hirochiyo

    1981-01-01

    Since the introduction of CT in February, 1977, 64 cases with 67 traumatic extradural hematomas were experienced. These cases were analysed, especially with regard to CT findings of the hematomas, small extradural hematomas, and combined intracerebral hematomas, and the correlation between CT gradings and clinical outcomes. From these analyses, the following results were obtained. In 64 out of 66 hematomas, except for a case of sagittal sinus hematoma, shapes were biconvex on CT scans. The other two were planconvex and crescent. One sagittal sinus hematoma could not be diagnosed by CT alone because the highest parietal slice was not taken. In 60 acute hematomas, densities of 59 were high. The other one was hypo- or iso-dense. In six subacute hematomas, two were hypo- and iso-dense respectively. These two cases showed a marked dural enhancement for contrast material. Initial admission CT scans disclosed 35 large hematomas (> 20 mm in thickness) and 29 small ones (<= 20 mm). The other three were not clear in the initial CT scans. Among 12 small hematomas for which initial CT scans were performed within 6 hours after injury and sequential CT scans were carried out, six were shown to become larger. Three of these were operated on thereafter. Eleven cases with small hematomas on the initial CT scans were operated on. Their bleeding sources were the middle meningeal arteries in three cases, dural vein in one, fracture sites in four, and unclear in three. In 16 combined intradural lesions, ten were traumatic intracerebral hematomas. Four of these intracerebral hematomas were found simultaneously with extradural ones in CT scans. Six were obviously found later than the latter. Two cases with multiple extradural hematomas were reported. After evacuation of one extradural hematoma, additional extradural and intracerebral hematomas developed at other sites in these cases. (author)

  9. Delayed Posttraumatic Subacute Lumbar Subarachnoid Hematoma: Case Report and Review of the Literature.

    Science.gov (United States)

    Picart, Thiébaud; Jacquesson, Timothée; Jouanneau, Emmanuel; Berhouma, Moncef

    2018-05-01

    Traumatic spinal subarachnoid hematoma, associated or not with a concurrent subdural hematoma, has rarely been described. The evolution of such hematomas is heterogeneous. This study aims at defining the most accurate management, which is currently not standardized. A 20-year-old man, victim of a high-kinetic road accident 5 days before and with several nonneurologic nonsurgical vertebral fractures, experienced a sudden dorsolumbar pain radiating to his lower limbs. A rapidly progressive asymmetric paraparesis with loss of reflexes was noticed, associated with bilateral global hypoesthesia of the lower limbs and with acute urinary retention, whereas the anal tonicity was preserved (American Spinal Injury Association C). Magnetic resonance imaging scan revealed a conus medullaris compression at the level of the L1-L2 vertebrae by an intradural expansive mass. Immediate surgical decompression revealed a strictly subarachnoid hematoma. Venous bleeding was seen at the level of the conus medullaris and controlled. Pathologic examination of the clot excluded an underlying tumor or vascular abnormality. The complete coagulation profile was normal. Six weeks after surgery, the neurologic examination revealed only a slight tactile hypoesthesia of the left thigh. With only 4 reported cases, purely subarachnoid spinal hematomas remain widely rarer than epidural hematomas. The reported case possesses a certain number of peculiarities: young age, pure subarachnoid location, lumbar location, occurrence after a car accident, subacute onset, and excellent neurologic recovery. In our opinion, a symptomatic subarachnoid spinal hematoma should be surgically evacuated at the early phase so neurologic recovery can be expected. Copyright © 2018 Elsevier Inc. All rights reserved.

  10. Estudo comparativo entre os diagnósticos mielográfico anátomo-cirúrgico nas afecções raquemedulares

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    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.

  11. 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.)

  12. MRI Findings of Early-Stage Hyperacute Hemorrhage Causing Extramedullary Compression of the Cervical Spinal Cord in a Dog with Suspected Steroid-Responsive Meningitis-Arteritis

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    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.

  13. 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

  14. 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

  15. Navigation and Robotics in Spinal Surgery: Where Are We Now?

    Science.gov (United States)

    Overley, Samuel C; Cho, Samuel K; Mehta, Ankit I; Arnold, Paul M

    2017-03-01

    Spine surgery has experienced much technological innovation over the past several decades. The field has seen advancements in operative techniques, implants and biologics, and equipment such as computer-assisted navigation and surgical robotics. With the arrival of real-time image guidance and navigation capabilities along with the computing ability to process and reconstruct these data into an interactive three-dimensional spinal "map", so too have the applications of surgical robotic technology. While spinal robotics and navigation represent promising potential for improving modern spinal surgery, it remains paramount to demonstrate its superiority as compared to traditional techniques prior to assimilation of its use amongst surgeons.The applications for intraoperative navigation and image-guided robotics have expanded to surgical resection of spinal column and intradural tumors, revision procedures on arthrodesed spines, and deformity cases with distorted anatomy. Additionally, these platforms may mitigate much of the harmful radiation exposure in minimally invasive surgery to which the patient, surgeon, and ancillary operating room staff are subjected.Spine surgery relies upon meticulous fine motor skills to manipulate neural elements and a steady hand while doing so, often exploiting small working corridors utilizing exposures that minimize collateral damage. Additionally, the procedures may be long and arduous, predisposing the surgeon to both mental and physical fatigue. In light of these characteristics, spine surgery may actually be an ideal candidate for the integration of navigation and robotic-assisted procedures.With this paper, we aim to critically evaluate the current literature and explore the options available for intraoperative navigation and robotic-assisted spine surgery. Copyright © 2016 by the Congress of Neurological Surgeons.

  16. Pediatric intraspinal neoplasms; Paediatrische intraspinale Neoplasien

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    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

  17. 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.

  18. 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

  19. 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

    2018-04-01

    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

  20. Pulsed laser-induced liquid jet: evolution from shock/bubble interaction to neurosurgical application

    Science.gov (United States)

    Nakagawa, A.; Kumabe, T.; Ogawa, Y.; Hirano, T.; Kawaguchi, T.; Ohtani, K.; Nakano, T.; Sato, C.; Yamada, M.; Washio, T.; Arafune, T.; Teppei, T.; Atsushi, K.; Satomi, S.; Takayama, K.; Tominaga, T.

    2017-01-01

    The high-speed liquid (water) jet has distinctive characteristics in surgical applications, such as tissue dissection without thermal damage and small blood vessel preservation, that make it advantageous over more conventional instruments. The continuous pressurized jet has been used since the first medical application of water jets to liver surgery in the 1980s, but exhibited drawbacks partly related to the excess water supply required and unsuitability for application to microsurgical instruments intended for deep, narrow lesions (endoscopic instrumentation and catheters) due to limitations in miniaturization of the device. To solve these issues, we initiated work on the pulsed micro-liquid jet. The idea of the pulsed micro-liquid jet originated from the observation of tissue damage by shock/bubble interactions during extracorporeal shock wave lithotripsy and evolved into experimental application for recanalization of cerebral embolisms in the 1990s. The original method of generating the liquid jet was based on air bubble formation and microexplosives as the shock wave source, and as such could not be applied clinically. The air bubble was replaced by a holmium:yttrium-aluminum-garnet (Ho:YAG) laser-induced bubble. Finally, the system was simplified and the liquid jet was generated via irradiation from the Ho:YAG laser within a liquid-filled tubular structure. A series of investigations revealed that this pulsed laser-induced liquid jet (LILJ) system has equivalent dissection and blood vessel preservation characteristics, but the amount of liquid usage has been reduced to less than 2 μ l per shot and can easily be incorporated into microsurgical, endoscopic, and catheter devices. As a first step in human clinical studies, we have applied the LILJ system for the treatment of skull base tumors through the transsphenoidal approach in 9 patients (7 pituitary adenomas and 2 chordomas), supratentorial glioma (all high grade glioma) in 8 patients, including one with

  1. Proton radiotherapy in management of pediatric base of skull tumors

    International Nuclear Information System (INIS)

    Hug, Eugen B.; Sweeney, Reinhart A.; Nurre, Pamela M.; Holloway, Kitty C.; Slater, Jerry D.; Munzenrider, John E.

    2002-01-01

    Purpose: Primary skull base tumors of the developing child are rare and present a formidable challenge to both surgeons and radiation oncologists. Gross total resection with negative margins is rarely achieved, and the risks of functional, structural, and cosmetic deficits limit the radiation dose using conventional radiation techniques. Twenty-nine children and adolescents treated with conformal proton radiotherapy (proton RT) were analyzed to assess treatment efficacy and safety. Methods and Materials: Between July 1992 and April 1999, 29 patients with mesenchymal tumors underwent fractionated proton (13 patients) or fractionated combined proton and photon (16 patients) irradiation. The age at treatment ranged from 1 to 19 years (median 12); 14 patients were male and 15 female. Tumors were grouped as malignant or benign. Twenty patients had malignant histologic findings, including chordoma (n=10), chondrosarcoma (n=3), rhabdomyosarcoma (n=4), and other sarcomas (n=3). Target doses ranged between 50.4 and 78.6 Gy/cobalt Gray equivalent (CGE), delivered at doses of 1.8-2.0 Gy/CGE per fraction. The benign histologic findings included giant cell tumors (n=6), angiofibromas (n=2), and chondroblastoma (n=1). RT doses for this group ranged from 45.0 to 71.8 Gy/CGE. Despite maximal surgical resection, 28 (97%) of 29 patients had gross disease at the time of proton RT. Follow-up after proton RT ranged from 13 to 92 months (mean 40). Results: Of the 20 patients with malignant tumors, 5 (25%) had local failure; 1 patient had failure in the surgical access route and 3 patients developed distant metastases. Seven patients had died of progressive disease at the time of analysis. Local tumor control was maintained in 6 (60%) of 10 patients with chordoma, 3 (100%) of 3 with chondrosarcoma, 4 (100%) of 4 with rhabdomyosarcoma, and 2 (66%) of 3 with other sarcomas. The actuarial 5-year local control and overall survival rate was 72% and 56%, respectively, and the overall survival

  2. 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.

  3. 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 individ