Sample records for chordoma

  1. Sellar chondroid chordoma

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    Xia LI


    Full Text Available Objective To investigate the clinical and pathological characteristics of sellar chondroid chordoma and discuss differential diagnosis of sellar chondroid chordoma with literature review. Methods The clinical manifestations of a patient with chondroid chordoma occurring in sellar area were stated. The morphological characteristics and immune phenotype were analyzed by using HE and immunohistochemical staining, and related literatures were reviewed. Results A 66-year-old male mainly presented with repeated hyponatremia. MRI revealed a well-circumscribed, round and space-occupying mass in sellar area. The tumor was removed under endoscope via the right nasal cavity. During the resection, the tumor could be seen locating in sellar region with solid, tough quality and clear boundaries. Histologically, part of the tumor showed the chondroid differentiation in classical chordoma. The immunohistochemistry of this tumor was positive for cytokeratin (CK, epithelial membrane antigen (EMA and S-100 protein (S-100, and Ki-67 labeling index was about 1%. The pathological diagnosis was sellar chondroid chordoma. During the follow-up period of 11 months, the patient was in good condition and no tumor recurrence was found. Conclusions Despite low incidence, chondroid chordoma usually develops in the midline regions with distinctive histological features and immunohistochemical phenotypes. In general, the prognosis is better than general type of chordoma, and the diagnosis should be differentiated from general types of chordoma and chondrosarcoma.doi:10.3969/j.issn.1672-6731.2014.02.007

  2. Vaccine Therapy for Unresectable Chordoma (United States)

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


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    Full Text Available "Chondroid chordoma" is a controversial and confusing entity, a biphasic malignant neoplasm possessing elements of both chordoma and cartilaginous tissue. The annual incidence of chordoma is approximately one in one million. Chondroid chordoma is a subtype of chordoma possessing elements of both chordoma and cartilaginous tissue with better prognosis than classic (non chondroid chordomas. We have come across a case of Chondroid Chordoma in a 55 year old male. Our report features particularly a rare entity, chondroid chordoma, with an exceptional localization to the nasal septum. Chondroid Chordomas are likely to recur and hence diagnostically important to institute effective treatment

  4. 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)


    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.

  5. Chordoma in the sella turcica. (United States)

    Kakuno, Yoshiteru; Yamada, Takashi; Hirano, Hiroshi; Mori, Hiroshi; Narabayashi, Isamu


    A 75-year-old man presented with a rare case of chordoma in the sella turcica of the skull base. He had been treated for hypertension and chronic renal failure since 1990. Computed tomography detected a tumor in the sella turcica in 1994, but the patient had no clinical complaints and the serum pituitary hormone levels were normal. He died of disseminated intravascular coagulation, myocardial infarction, pulmonary infection, and multiple cerebral infarctions in 2000. At autopsy, the tumor in the sella turcica was 3.1 cm in greatest diameter and had compressed the pituitary gland posteriorly. Histological examination found oval cells and vacuolated short spindle-shaped cells which showed morphological changes similar to myxoma cells. The tumor was lobulated by narrow connective tissues. The tumor did not contain any cartilaginous tissue components, and was stained positively for epithelial membrane antigen but negatively for S-100 protein. The final diagnosis was chordoma. There was no association between the tumor and the cause of death.

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

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    Kamel, Mahmoud Hamdy


    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.

  7. Frequent activation of EGFR in advanced chordomas

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    Dewaele Barbara


    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. Pudendal Neuralgia as the Initial Manifestation of Infiltrative Sacrococcygeal Chordoma (United States)

    Carrasco García de León, Sira; Flores Barragán, José Manuel; Villasanti Rivas, Natalia


    Sacrococcygeal chordoma is a malignant tumour originating from remnants of the notochord. Chordomas are slow-growing tumours whose symptoms develop insidiously. We present the case of a 72-year-old woman with a 6-month history of genital pain radiating to the perianal area and exacerbating when she was in a sitting position. MRI and PET studies revealed a large mass in the sacrococcygeal region causing bone destruction and invasion of neurovascular structures. The immunohistochemical study of the surgical specimen determined it to be chordoma. This is the first published case of pudendal neuralgia as a form of presentation of sacrococcygeal chordoma. PMID:27721781

  9. Pudendal Neuralgia as the Initial Manifestation of Infiltrative Sacrococcygeal Chordoma

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    Sira Carrasco García de León


    Full Text Available Sacrococcygeal chordoma is a malignant tumour originating from remnants of the notochord. Chordomas are slow-growing tumours whose symptoms develop insidiously. We present the case of a 72-year-old woman with a 6-month history of genital pain radiating to the perianal area and exacerbating when she was in a sitting position. MRI and PET studies revealed a large mass in the sacrococcygeal region causing bone destruction and invasion of neurovascular structures. The immunohistochemical study of the surgical specimen determined it to be chordoma. This is the first published case of pudendal neuralgia as a form of presentation of sacrococcygeal chordoma.

  10. Novel targeted therapies in chordoma: an update

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    Di Maio S


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

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

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    Roberto Jose Diaz


    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.

  12. Sacrococcygeal chordoma: MR imaging in 30 patients

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    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)


    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.)

  13. Clival chordoma; CT and MR findings

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    Lee, Si Kyung; Han, Chun Hwan; Lee, Moon Ok; Kim, Mie Young; Yi, Jeong Geun; Lee, Joo Hyuk [Kang Nam General Hospital Pubic Corporation, Seoul (Korea, Republic of); Bae, Sang Hoon [Hallym University College of Medicine, Seoul (Korea, Republic of)


    A retrospectively analysis of CT and MR findings was performed in five patients with histologically proved chordoma including one with chondroid chordoma. All tumors were mostly isodense to gray matter on unenhanced CT, and the single intensities were iso or low and high on T1 and T2-weighted MR images, respectively. The tumors had an increase in their density on enhanced CT and MR in four patients, but a chondroid chordoma was poorly enhanced after injection of contrast medicum on CT. Four tumors contained calcifications in CT images and two lesionsshowed hemorrhage in MR images. Cavenous sinus was involved in all patients, and brain stem and basilar artery were compressed by the tumors in three cases. Pituitary gland was only displaced upward in three patients. Clivus was destroyed in all cases, and sella turcica and sphenoid bone were involved in three patients. CT is better than MR in demonstrating calcifications and bone destruction. In defining the extension of tumor, MR appears to be superior to CT in evaluation of the relationship between the tumor and the surrounding structures.

  14. Intradural chordoma of the Meckel's cave: a challenging differential diagnosis. (United States)

    Barresi, Valeria; Caffo, Maria; Alafaci, Concetta; Granata, Francesca; Tuccari, Giovanni


    Chordomas are midline tumors that arise from embryonic remnants of the notochord and are considered to be malignant tumors because of their tendency to invade and destroy the involved bone. Cases of intradural chordomas without bone involvement have been rarely described with a predilection for prepontine location. The absence of bony invasion renders the complete excision of these tumors more feasible and is related to their better prognosis in comparison to conventional chordomas. Herein we report the first intradural chordoma arising in the Meckel's cave. The intradural location of the lesion, outside midline structures, in the absence of bone infiltration, made the differential diagnosis versus other meningeal lesions such as chordoid meningioma challenging. The intense and strong immunohistochemical expression of pan-cytokeratins, S100, cytokeratin-19 and of the notochordal marker brachyury allowed differential diagnosis toward other tumors showing chordoid morphology. The expression of brachyury, which had not been previously analyzed in intradural chordoma, definitely links the histogenesis of this neoplasia to the notochord, similar to that of conventional chordoma. We also show that, different from conventional chordoma, intradural chordoma does not express the metallo-proteinases (MMPs) -2 and -9, which may account for its indolent biological behavior.

  15. Aspiration cytology of metastatic chordoma. A case report. (United States)

    Elliott, E C; McKinney, S; Banks, H; Fulks, R M


    A patient with previously diagnosed sacrococcygeal chordoma presented with multiple skin nodules. Cytologic examination of an aspirate from one of these nodules showed syncytial clusters of hyperchromatic cells surrounded by extracellular mucin. The characteristic physaliphorous cells, although present in a biopsy of the primary sacrococcygeal tumor, were not observed in the aspirate or on histopathologic examination of three excised skin metastases. That chordoma metastases may lack physaliphorous cells should be recognized.

  16. Infrasellar craniopharyngioma mimicking a clival chordoma : a case report.

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    Kachhara R


    Full Text Available An unusual case of entirely infrasellar craniopharyngioma mimicking a clival chordoma is described. Only 22 cases of craniopharyngioma with nasopharyngeal extension have been reported in the literature. Of the reported cases, most were primarily intracranial with secondary downward extension; only two were thought to originate from an infrasellar location. The present case is another example of an entirely infrasellar craniopharyngioma, with extensive clival destruction, mimicking a clival chordoma. Relevant literature on the subject is reviewed.

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

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    Sepidbakht Sepideh


    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.

  18. Chordomas: Is It Possible to Predict Recurrence?

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    Banu SARSIK


    Full Text Available Objective: Chordoma is a rare tumor with an unpredictable behaviour, and can display malignant behavior because of its tendency to local invasion and recurrence. We searched the prognostic value of histologic features, growth pattern, localization and Ki-67 proliferation index to predict disease-free survival.Material and Method: Twenty-nine cases diagnosed in a single center were included in the study and evaluated with their 81 surgical specimens, (29 primary tumors, 47 recurrent lesions and five metastatic foci regarding their matrix formation (myxoid, chondroid, cellular features, (pleomorphism, necrosis, inflammatory infiltration, patterns of proliferation (solid, trabecular, mixed and Ki-67 proliferation indices.Results: Eleven of the cases were females (37.9% while 18 of them were males (62.1% with a mean age of 54.1±14.6 (ranged between age 23-78 years. Thirty-eight percent of tumors were located in sacrococcigeal region followed by skull base and vertebrae (31% for both. Skull base chordomas which occured in younger patients (p=0.048 showed more trabecular pattern (p=0.04, chondroid matrix (p=0.063, lower Ki-67 (p=0.146 and longer disease-free survival (p=0.021. In contrast, tumors located in vertebrae, showed more “atypical” morphology with solid pattern, nuclear pleomorphism and dedifferentiation, higher Ki-67 indices and shorter disease-free survivals (p=0.021. Sacral tumors were the “intermediate group” which occurred in older patients, and demonstrated average Ki-67 proliferation indices and disease-free survivals.Conclusion: Vertebral localization, probably in relation with both histologic features and failure of surgery, appeared as a significant risk factor for recurrence and Ki-67 proliferation index retained its potential to predict disease-free survival.

  19. Intracranial chordoma presenting as acute hemorrhage in a child: Case report and literature review

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    Kenneth A Moore


    Conclusion: There are few previous reports of petroclival chordomas causing acute intracranial hemorrhage. To the authors′ knowledge, this is the first case of a petroclival chordoma presenting as acute intracranial hemorrhage in a pediatric patient. Although uncommon, it is important to consider chordoma when evaluating a patient of any age presenting with a hemorrhagic lesion of the clivus.

  20. Paraspinal chordoma mimicking a neurofibroma: a rare but important radiological pitfall

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    Kotnis, Nikhil; Goepel, John [Royal Hallamshire Hospital, Sheffield Teaching Hospitals, Sheffield (United Kingdom)


    We present an unusual case of a chordoma presenting as an extradural spinal tumour with extension through an expanded intervertebral foramen to form a large paraspinal mass. The magnetic resonance imaging appearances closely mimicked a neurofibroma; however, pre-operative biopsy confirmed the diagnosis of chordoma. This is, to our knowledge, the tenth reported case of chordoma presenting as a mass expanding the intervertebral foramen. Thus, while it is a rare form of chordoma, it can lead to a recognised radiological pitfall. Making the distinction from neurofibroma before surgery is essential, as radical dissection of chordoma is required to reduce the risk of local recurrence. (orig.)

  1. Chondroid chordoma of the sella turcica mimicking a pituitary adenoma. (United States)

    Wu, Arthur W; Bhuta, Sunita; Salamon, Noriko; Martin, Neil; Wang, Marilene B


    We report an unusual case of a chondroid chordoma of the sella turcica that mimicked the clinical and radiologic presentation of the more common pituitary adenoma. A 50-year-old man presented with bitemporal visual field deficits. Magnetic resonance imaging (MRI) detected a sellar mass that was suggestive of a pituitary adenoma. However, the intraoperative appearance of the mass was not consistent with an adenoma, and frozen-section pathology was obtained. Pathology identified the mass as a malignant lesion. Based on this finding, the mass was treated more aggressively. Chondroid chordomas are rare and slowly growing but locally aggressive tumors. The prognosis depends on the ability to totally resect the mass, so differentiating this tumor from a benign lesion is critical. An intrasellar chordoma can be confused clinically and radiologically with a pituitary adenoma. These two lesions are nearly identical on MRI, although T2-weighted imaging sometimes demonstrates higher intensity with a chondroid chordoma. Computed tomography may be helpful in demonstrating bony destruction by these lesions, as can the presence of intralesional calcifications. Intraoperative findings of bony invasion or a purple-red color may also lead the surgeon to suspect a diagnosis other than pituitary adenoma.

  2. Computer Navigation-aided Resection of Sacral Chordomas

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    Yong-Kun Yang


    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.

  3. Characteristics and Patterns of Metastatic Disease from Chordoma

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    Victoria A. Young


    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.

  4. Chordoma. Report on treatment results in eighteen cases

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    Lybeert, M.L.M.; Meerwaldt, J.H.


    Eighteen patients with a proven histologic diagnosis of chordoma were treated between 1949 and 1982. Four patients received only surgery, 4 patients only radiation therapy, and 10 patients received surgery and postoperative radiation therapy to a varying dose. The results suggest that a higher radiation dose gives longer recurrence-free survival, and that the best long term results can be achieved by combining surgery - as radically as possible - with radiation therapy to a dose level of 60 to 65 Gy. In view of the number of marginal recurrences (2 out of the 14 patients who received radiation therapy), the importance of choosing the right treatment volume is stressed.

  5. 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)


    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.)

  6. Intradural chordoma mimicking a lateral sphenoid wing meningioma: a case report. (United States)

    Kunert, Przemysław; Dziedzic, Tomasz; Matyja, Ewa; Marchel, Andrzej


    Chordomas are rare tumours arising from notochordal remnants. Classical chordomas are generally extradural and, despite benign histopathology, they typically destroy the clivus and surrounding bone structures. Intradural lesions are extremely rare and less than thirty cases of intracranial, exclusively intradural chordomas have been reported so far. The intracranial, intradural but extranotochordal location of chordoma is extremely unique. The authors present a case of chordoma in intracranial location that clinically mimics lateral sphenoid wing meningioma. A previously healthy 39-year-old man was admitted to our Department because of optic disc oedema without neurological deficits. Neuroimaging studies showed a large, contrast-enhanced tumour in the right frontotemporal region that was thought to be a pterional meningioma. The patient underwent successful removal of the tumour. Histopathological study revealed a typical pattern of chordoma, confirmed by immunohistochemical findings. Because of the tumour location the differentiation between chordoma and chordoid meningioma ought to be considered. Such cases, including the present one, may lead to the conclusion that embryonic notochordal remnants may be lost in different places, even away from the neuroaxis.

  7. Intradural spinal seeding and fatal progression of a sacrococcygeal chordoma:a case report

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    JI Tao; GUO Wei; SHEN Dan-hua; YANG Yi; TANG Shun


    @@ Chordoma as a rare malignant tumor arising from remnants of the fetal notochord accounts for 1%-4% of primary bone tumors.It is usually predominant in males.Local recurrences are common (44%-78%)1-3 and distant metastases may occur years after the initial presentation.The reposed rates of metastases range from 10% to 48%,4-6 which are usually accompanied by a sacrococcygeal chordoma.Cases of surgical seeding and intradural spinal seeding also have been reported clinically.7-9 To our knowledge,the present case is the first one concerning cerebrospinal fluid metastases and fatal progression of a sacrococcygeal chordoma.

  8. Multilevel oblique corpectomies as an effective surgical option to treat cervical chordoma in a young girl. (United States)

    Delfini, Roberto; Marruzzo, Daniele; Tarantino, Roberto; Marotta, Nicola; Landi, Alessandro


    Chordomas are malignant tumors arising from notochordal remnants. They are the most frequent tumors of the spine after plasmacytomas. Only 6% of chordomas are localized to the cervical level. In young patients, chordomas are rare and unpredictable. Despite this, the treatment of choice remains the total resection, as much as possible, followed by proton beam radiation. This case was managed using a precarotid and retrocarotid approach at the same time. The tumor was completely resected with the edges free from disease. The cervical spine was stabilized with an anterior plating C2-C4. Eighteen months after surgery the patient is still free from illness. Multilevel oblique corpectomies are an available and safe option for the treatment of upper cervical chordomas.

  9. Metastatic Chordoma: Report of the Two Cases and Review of the Literature (United States)

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


    Chordomas are rare malignant bone tumours with a predilection for the axial skeleton, especially the sacrum and skull base. Median survival in patients with metastatic disease is usually dismal. Treatment is challenging due to the propensity for local recurrence, metastatic disease as well as lack of clear consensus regarding the optimal management. Our case report highlights two cases of sacral chordoma with locally recurrent and widespread metastatic disease, stable on molecular targeted therapy. PMID:26180502

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



    Background: Chordomas are rare malignant tumors that are difficult to treat and have high recurrence rates despite aggressive therapy. Objectives: We present the first case of a patient with a clival chordoma in which complete tumor ablation was achieved using Magnetic Resonance guided Laser Induced Thermal Therapy (LITT) delivered via an endoscopic endonasal approach. We analyzed the safety and feasibility of this approach and quantified the response of this pathology to thermal energy. T...

  11. Proton Therapy for Reirradiation of Progressive or Recurrent Chordoma

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    McDonald, Mark W., E-mail: [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)


    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

  12. The FGFR/MEK/ERK/brachyury pathway is critical for chordoma cell growth and survival. (United States)

    Hu, Yunping; Mintz, Akiva; Shah, Sagar R; Quinones-Hinojosa, Alfredo; Hsu, Wesley


    Recent evidence suggests that the expression of brachyury is necessary for chordoma growth. However, the mechanism associated with brachyury-regulated cell growth is poorly understood. Fibroblast growth factor (FGF), a regulator of brachyury expression in normal tissue, may also play an important role in chordoma pathophysiology. Using a panel of chordoma cell lines, we explored the role of FGF signaling and brachyury in cell growth and survival. Western blots showed that all chordoma cell lines expressed fibroblast growth factor receptor 2 (FGFR2), FGFR3, mitogen-activated protein kinase kinase (MEK) and extracellular signal-regulated kinase (ERK), whereas no cell lines expressed FGFR1 and FGFR4. Results of enzyme-linked immunosorbent assay indicated that chordoma cells produced FGF2. Neutralization of FGF2 inhibited MEK/ERK phosphorylation, decreased brachyury expression and induced apoptosis while reducing cell growth. Activation of the FGFR/MEK/ERK/brachyury pathway by FGF2-initiated phosphorylation of FGFR substrate 2 (FRS2)-α (Tyr196) prevented apoptosis while promoting cell growth and epithelial-mesenchymal transition (EMT). Immunofluorescence staining showed that FGF2 promoted the translocation of phosphorylated ERK to the nucleus and increased brachyury expression. The selective inhibition of FGFR, MEK and ERK phosphorylation by PD173074, PD0325901 and PD184352, respectively, decreased brachyury expression, induced apoptosis, and inhibited cell growth and EMT. Moreover, knockdown of brachyury by small hairpin RNA reduced FGF2 secretion, inhibited FGFR/MEK/ERK phosphorylation and blocked the effects of FGF2 on cell growth, apoptosis and EMT. Those findings highlight that FGFR/MEK/ERK/brachyury pathway coordinately regulates chordoma cell growth and survival and may represent a novel chemotherapeutic target for chordoma.

  13. Upregulation of metastasis-associated PRL-3 initiates chordoma in zebrafish. (United States)

    Li, Li; Shi, Hongshun; Zhang, Mingming; Guo, Xiaoling; Tong, Fang; Zhang, Wenliang; Zhou, Junyi; Wang, Haihe; Yang, Shulan


    The metastasis-associated phosphatase of regenerating liver-3 (PRL-3) plays multiple roles in progression of various human cancers; however, significance of its role during development has not been addressed. Here we cloned and characterized the expression pattern of zebrafish prl-3 transcript and showed that it is ubiquitiously expressed in the first 24 h of development with both maternal and zygotic expressions. The transcripts become progressively restricted to the notochord, vessels and the intestine by 96 h post-fertilization. Notably, overexpression of zebrafish Prl-3 (zPrl-3) and human PRL-3 induces notochord malformation in zebrafish. This phenotype resembles chordoma and is confirmed by associated misexpression of notochord-specific markers. Clinical significance of the PRL-3 in chordoma is strongly suggested by detection of PRL-3 antigen in clinical chordoma specimens. Collectively, our results uncovered that aberrant overexpression of PRL-3 could initiate chordoma in early development and suggest the use of PRL-3 could be used as a predictor and a therapeutic target for chordoma.

  14. 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


    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.

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

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    James Barrese


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

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


    Object 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. Methods 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. Results 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 < 0.001), spinal location (p = 0.018), and if the patient received adjuvant therapy (p < 0.001) were significantly different between the 2 cohorts. Overall, 58 (35%) patients developed local

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

    Institute of Scientific and Technical Information of China (English)

    Jun-qi Liu; Qiu-hang Zhang; Zhen-lin Wang


    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).Significantly elevated MIB-1 LI was also detected in skull base chondrosarcoma tissues, while there was negative expression of p16, cyclin D1 and Rb (P < 0.05).In skull base chordoma, p16 negatively correlated with cyclin D1 and Rb, while cyclin D1 positively correlated with Rb.Additionally, p16, cyclin D1, Rb, or MIB-1 expression showed no correlation with age, gender, or pathological classification of patients with skull base chordoma (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.

  18. EGFR inhibitors identified as a potential treatment for chordoma in a focused compound screen. (United States)

    Scheipl, Susanne; Barnard, Michelle; Cottone, Lucia; Jorgensen, Mette; Drewry, David H; Zuercher, William J; Turlais, Fabrice; Ye, Hongtao; Leite, Ana P; Smith, James A; Leithner, Andreas; Möller, Peter; Brüderlein, Silke; Guppy, Naomi; Amary, Fernanda; Tirabosco, Roberto; Strauss, Sandra J; Pillay, Nischalan; Flanagan, Adrienne M


    Chordoma is a rare malignant bone tumour with a poor prognosis and limited therapeutic options. We undertook a focused compound screen (FCS) against 1097 compounds on three well-characterized chordoma cell lines; 154 compounds were selected from the single concentration screen (1 µm), based on their growth-inhibitory effect. Their half-maximal effective concentration (EC50 ) values were determined in chordoma cells and normal fibroblasts. Twenty-seven of these compounds displayed chordoma selective cell kill and 21/27 (78%) were found to be EGFR/ERBB family inhibitors. EGFR inhibitors in clinical development were then studied on an extended cell line panel of seven chordoma cell lines, four of which were sensitive to EGFR inhibition. Sapitinib (AstraZeneca) emerged as the lead compound, followed by gefitinib (AstraZeneca) and erlotinib (Roche/Genentech). The compounds were shown to induce apoptosis in the sensitive cell lines and suppressed phospho-EGFR and its downstream pathways in a dose-dependent manner. Analysis of substituent patterns suggested that EGFR-inhibitors with small aniline substituents in the 4-position of the quinazoline ring were more effective than inhibitors with large substituents in that position. Sapitinib showed significantly reduced tumour growth in two xenograft mouse models (U-CH1 xenograft and a patient-derived xenograft, SF8894). One of the resistant cell lines (U-CH2) was shown to express high levels of phospho-MET, a known bypass signalling pathway to EGFR. Neither amplifications (EGFR, ERBB2, MET) nor mutations in EGFR, ERBB2, ERBB4, PIK3CA, BRAF, NRAS, KRAS, PTEN, MET or other cancer gene hotspots were detected in the cell lines. Our findings are consistent with the reported (p-)EGFR expression in the majority of clinical samples, and provide evidence for exploring the efficacy of EGFR inhibitors in the treatment of patients with chordoma and studying possible resistance mechanisms to these compounds in vitro and in vivo. © 2016

  19. Sacrococcygeal chordoma in infancy showing an aggressive clinical course: an autopsy case report. (United States)

    Shinmura, Yuichiro; Miura, Katsutoshi; Yajima, Shuhei; Tsutsui, Yoshihiro


    The autopsy case of a 3-year 6-month-old boy with chordoma arising in the sacrococcygeal region is presented. The primary lesion of the sacrococcygeal area was unresectable and lung metastasis was detected. He was treated with multi-agent systemic chemotherapy and radiation therapy, but the tumor was less responsive to these therapies. He died about one year after first admission. An autopsy revealed a massive sacrococcygeal mass and metastasis in the thoracic and lumbar vertebrae, retroperitoneal and mediastinal lymph nodes, and also in the bilateral lungs and liver. Histologically, the tumor was composed of 'pink' cells and scattered 'physaliphorous' cells with a myxoid matrix. Sacrococcygeal chordoma in infancy is very rare. Our case showed a highly aggressive clinical course.

  20. 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)


    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.)

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

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    Constantine L. Karras


    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.

  2. Clinical Value of Interventional Embolization for Sacral Chordoma before Surgical Treatment

    Institute of Scientific and Technical Information of China (English)

    Zhihui CHANG; Zhaoyu LIU; Jiahe Zheng; Zaiming LU; Qiyong GUO


    Objetive:To evaluate the efficacy of preoperative transcatheter arterial embolization in the treatment of sacral chordoma,and to explore the choice of embolization timing. Methods:32 patients underwent the posterior approach after TAE of the main arteries that supplied the sacral chordoma. Intraoperative bleeding amount of each patient was recorded and compared between-group differences at different operation times. Results: After embolization, 12 patients were received resection within 24 hours (group A),that of 10 cases between 24 ~ 48h (group B), of another 10 cases between 48~ 72h (group C). All of the 32 tumors were removed intact with intraoperative bleeding about (894±199)ml, without any shock or death,nor injuries on abdominal organs such as rectum and ureter. There was no statistical significance in tumor size among group A,B and C (P>0.05). Data gave statistical significance in intraoperative blood loss between group A and B (P<0.01) ,there was no statistical differences between group B and C (P>0.05), in spite of group B slightly less than group C. Conclusion: Preoperative TAE for excising the sacral tumor can significantly decrease intraoperative blood loss, make the surgical field clear,and facilitate the maximal removal of the sacral chordoma. It would be best to select the embolization timing within 24 hours before surgical operation.

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

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    Cláudio Régis S. Silveira


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

  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


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

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    Yamaguchi, Takehiko [Department of Anatomic and Diagnostic Pathology, Dokkyo University School of Medicine, Mibu, Tochigi (Japan); Yamato, Minoru [Department of Radiology, International Catholic Hospital, Tokyo (Japan); Saotome, Koichi [Department of Orthopaedic Surgery, Dokkyo University School of Medicine, Mibu, Tochigi (Japan)


    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.)

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

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    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


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

  7. Chordoma of the thoracic vertebrae in a Bengal tiger (Panthera tigris tigris). (United States)

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


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

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

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    Mirra, J.M. [Dept. of Orthopaedic Pathology, Orthopaedic Hospital, Los Angeles, CA (United States); Brien, E.W. [Dept. of Musculoskeletal Tumors, Orthopaedic Hospital, Los Angeles, CA (United States)


    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.)

  9. Transoral vs. endoscopic endonasal approach for clival/upper cervical chordoma. (United States)

    Shidoh, Satoka; Toda, Masahiro; Kawase, Takeshi; Nakajima, Hideo; Tomita, Toshiki; Ogawa, Kaoru; Yoshida, Kazunari


    The surgical results of 18 cases of clival/upper cervical chordoma treated in the last decade via the endoscopic endonasal approach (EEA, 9 cases) and the transoral-transpalatal approach (TO-TPA, 9 cases) were compared. Each group showed the same incidence of subdural invasion, with 5 cases each. The superior (frontal base) and lateral surgical fields were wider by EEA, but the inferior view lower than the cranio-vertebral junction (CVJ) was wider by TO-TPA. Gross total removal was achieved in 3 cases in the EEA group, but in only 1 case in the TO-TPA group. Differences in radicality might be due to the extent of the lateral and subdural overview. However for large tumors extending below the CVJ, TO-TPA was the only viable approach for surgical removal. Surgical complications were higher in the EEA (4 cases) than the TO-TPA group (1 case), and were mainly caused by aggressive management of subdural invasion in the EEA group. Post-operative oral intake was earlier and the operative time was shorter in the EEA group. The surgical results were more radical and less invasive in the EEA group than the TO-TPA group. However in tumors extending below the CVJ, the surgical field in EEA was limited, indicating the need to use the transoral route or a combination of routes. A higher complication rate following subdural management was a negative factor that requires improvement in the EEA group and two-staged EEA followed by a transcranial approach may be considered for the cases with subdural invasion.

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

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    Si, Ming-Jue, E-mail: [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: [Department of Radiology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025 (China); Yuan, Fei, E-mail: [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)


    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.

  11. In vitro characterization of cells derived from chordoma cell line U-CH1 following treatment with X-rays, heavy ions and chemotherapeutic drugs

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    Uesaka Mitsuru


    Full Text Available Abstract Background Chordoma, a rare cancer, is usually treated with surgery and/or radiation. However, very limited characterizations of chordoma cells are available due to a minimal availability (only two lines validated by now and the extremely long doubling time. In order to overcome this situation, we successfully derived a cell line with a shorter doubling time from the first validated chordoma line U-CH1 and obtained invaluable cell biological data. Method After isolating a subpopulation of U-CH1 cells with a short doubling time (U-CH1-N, cell growth, cell cycle distribution, DNA content, chromosome number, p53 status, and cell survival were examined after exposure to X-rays, heavy ions, camptothecin, mitomycin C, cisplatin and bleocin. These data were compared with those of HeLa (cervical cancer and U87-MG (glioblastoma cells. Results The cell doubling times for HeLa, U87-MG and U-CH1-N were approximately 18 h, 24 h and 3 days respectively. Heavy ion irradiation resulted in more efficient cell killing than x-rays in all three cell lines. Relative biological effectiveness (RBE at 10% survival for U-CH1-N was about 2.45 for 70 keV/μm carbon and 3.86 for 200 keV/μm iron ions. Of the four chemicals, bleocin showed the most marked cytotoxic effect on U-CH1-N. Conclusion Our data provide the first comprehensive cellular characterization using cells of chordoma origin and furnish the biological basis for successful clinical results of chordoma treatment by heavy ions.

  12. 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: [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)


    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.

  13. 颅底脊索瘤的影像诊断与鉴别论断%Imaging Diagnosis and Differential Diagnosis of Chordoma of the Skull Base

    Institute of Scientific and Technical Information of China (English)

    林颖; 游瑞雄; 江飞; 郑义浩


    目的:分析颅底脊索瘤的影像学特点,探讨CT与MR对颅底脊索瘤的诊断和鉴别论断价值。方法:回顾性分析33例经手术病理证实的颅底脊索瘤CT与MR表现。结果:颅底脊索瘤好发于斜坡与蝶鞍,在CT上主要表现为溶骨性骨质破坏,在MR上表现为T1WI上呈高低混杂信号, T2WI上以高信号为主的混杂信号。结论: CT在显示病灶的骨质破坏、病灶内残存的骨质及钙化灶方面优于MR,而MR在显示病灶的范围、对邻近结构的侵犯及与颅底重要结构的关系等方面明显优于CT,联合应用CT与MR检查,不仅可以提高颅底脊索瘤的诊断,还能为临床确定手术方式及手术入路提供重要的依据。%Objective: Analyze the image characteristics of chordoma of the skull base and discuss the diagnosis and differential di -agnosis value of CT and MRI.Methods: CT and MRI findings in 33 cases of pathologically proved chordoma were retrospectively ana-lyzed.Results: Chordoma of the skull base obliterans occur in clivus and sella turcica , Lesions mainly showed osteolytic bone destruc-tion on CT image, and were mixed signal on T1WI and hyper -intensity on T2WI.Conclusion: The residual sclerotin and calcifica-tion within the lesions were clearly depicted on CT.The extension of lesions , invasions of adjacent tissues and relationship with impor-tant structures of skull base were better displayed on MRI than CT.The combination of CT and MRI can improve diagnosis and provide important basis for operation method and route of chordoma.

  14. Chordoma-derived cell line U-CH1-N recapitulates the biological properties of notochordal nucleus pulposus cells. (United States)

    Fujita, Nobuyuki; Suzuki, Satoshi; Watanabe, Kota; Ishii, Ken; Watanabe, Ryuichi; Shimoda, Masayuki; Takubo, Keiyo; Tsuji, Takashi; Toyama, Yoshiaki; Miyamoto, Takeshi; Horiuchi, Keisuke; Nakamura, Masaya; Matsumoto, Morio


    Intervertebral disc degeneration proceeds with age and is one of the major causes of lumbar pain and degenerative lumbar spine diseases. However, studies in the field of intervertebral disc biology have been hampered by the lack of reliable cell lines that can be used for in vitro assays. In this study, we show that a chordoma-derived cell line U-CH1-N cells highly express the nucleus pulposus (NP) marker genes, including T (encodes T brachyury transcription factor), KRT19, and CD24. These observations were further confirmed by immunocytochemistry and flow cytometry. Reporter analyses showed that transcriptional activity of T was enhanced in U-CH1-N cells. Chondrogenic capacity of U-CH1-N cells was verified by evaluating the expression of extracellular matrix (ECM) genes and Alcian blue staining. Of note, we found that proliferation and synthesis of chondrogenic ECM proteins were largely dependent on T in U-CH1-N cells. In accordance, knockdown of the T transcripts suppressed the expression of PCNA, a gene essential for DNA replication, and SOX5 and SOX6, the master regulators of chondrogenesis. On the other hand, the CD24-silenced cells showed no reduction in the mRNA expression level of the chondrogenic ECM genes. These results suggest that U-CH1-N shares important biological properties with notochordal NP cells and that T plays crucial roles in maintaining the notochordal NP cell-like phenotype in this cell line. Taken together, our data indicate that U-CH1-N may serve as a useful tool in studying the biology of intervertebral disc. © 2016 The Authors. Journal of Orthopaedic Research Published by Wiley Periodicals, Inc. on behalf of Orthopaedic Research Society. J Orthop Res 34:1341-1350, 2016.

  15. [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: [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)


    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.

  16. 以突发性聋为首发表现的斜坡脊索瘤1例%A case of clivus chordoma with first appearance of sudden deafness

    Institute of Scientific and Technical Information of China (English)

    管华; 马志红; 郁葱颖


    The patient was hospitalized for sudden hearing impairment for one day. Physical examination: the blood pressure was 150/90 mm Hg, the tympanic membranes in both ears were complete and olopiesis. Audio-gram showed total deafness in the right ear and slight sensorineural deafness at speech frequency and 80 db for high tone air conduction and 70 db for bone conduction at high frequency in left ear. Tympanogram showed "A" type in both ears and the ipsilateral and contralateral acoustic reflex in both ears were not induced. BAEP showed that the V wave threshold on the right was not induced and it was 50 dbnHL on the left. CT showed a limited low density area in the clivus. MRI showed a space-occupying lesion behind the basilar clivus and ahead of brain stem. Pathological examination showed CK( +). EMA( +) , S-100( +) according to immunohistochemistry, which was in accordance with chondroid chordoma. Diagnosis: chondroid chordoma of clivus.

  17. 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: [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)


    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.

  18. 骶骨脊索瘤55例临床病理和免疫表型分析%Analysis of clinicopathologic features and immunophenotype in 55 patients with chordoma

    Institute of Scientific and Technical Information of China (English)

    张彤; 陈云新; 沈丹华; 王颖; 张银丽; 陈定宝; 宋秋静


    目的 探讨骶骨脊索瘤的临床病理特征,观察CK、vimentin、S-100蛋白及Ki-67在脊索瘤中的表达.方法 收集2007~2011年北京大学人民医院病理科存档的55例脊索瘤,分析其临床病理学特征,并采用免疫组化EnVision两步法检测CK、vimentin、S-100蛋白及Ki-67的表达.结果 55例骶骨脊索瘤患者中原发病例35例,复发病例20例,男女之比为2.2:1.发病年龄21~85岁,平均52.8岁.大部分病例主要呈结节状生长,局灶呈巢片状排列.肿瘤细胞主要由液滴状及星芒状细胞组成,细胞轻-中度异型,偶见核分裂,少部分病例可见坏死.免疫表型:CK、vimentin和S-100蛋白的阳性率分别为96.4%(53/55)、100%(55/55)、94.5%(52/55),Ki-67增殖指数为2%~25%.原发和继发的脊索瘤中CK、vimentin、S-100及Ki-67的表达无明显差异(P>0.05).结论 骶骨脊索瘤是一种易复发的低度恶性肿瘤,CK、vimentin、S-100蛋白结合Ki-67的免疫组化标记可辅助诊断脊索瘤.%Purpose To investigate the clinicopathologic features and the expression of CK, vimentin, S-100 and Ki-67 on patients with sacral chordoma. Methods 55 cases diagnosed as chordoma with available slides and clinical information, coded between 2007 and 2011, were retrieved from the our department of pathology. The expression of CK, vimentin, S-100 and Ki-67 were examined using immunohistochemistry. Results Of the 55 cases, 35 were primary and 20 were recurrent cases. The ratio of males to females is 2. 2 ? 1. The age ranged from 21 to 85 years ( median, 52. 8 years ). The majority of the tumor showed nodular growth pattern, focally with nests. The neoplasm were composed of physaliphorous cells and satellite-like cells. The tumor cells revealed mild to moderate atypia and the mitosis was rare. The focal necrosis was observed in a minority of cases. Immunohistochemistry was performed in 55 chordoma cases including 35 primary and 20 secondary cases. Immunohistochemical finding

  19. Imaging features of chordoma in the rare sites of spine (report of 7 cases)%发生于脊柱少见部位脊索瘤的影像学表现(附7例报告)

    Institute of Scientific and Technical Information of China (English)

    朱峰; 崔学锋; 李常城


    目的 了解发生于脊柱少见部位(颈椎、腰椎)脊索瘤的CT及MRI表现.方法 分析7例经病理证实的发生于颈椎、腰椎脊索瘤的CT和MRI表现.结果 7例脊索瘤,颈椎5例,腰椎2例,相邻多节段椎体受累,胸椎未见发生.脊椎脊索瘤表现为椎体轻度膨胀性骨质破坏,附件多受累及椎体周围相对明显分叶状软组织肿块,肿块CT值低于肌肉软组织,MRI呈明显长T1长T2信号,信号多不均匀.结论 脊索瘤具有较特征性CT及MR表现,结合增强扫描能够很好的评价脊索瘤的部位、范围、形态及对周围组织侵犯情况.%Objective To investigate the CT and MRI imaging features of chordoma in the rare sites of spine (cervical and lumbar vertebra).Methods 7 cases with chordoma in the cervical and lumbar spine verified by pathology were analyzed.Results Of the seven cases,five cases were found in cervical vertebra and two cases in lumbar vertebra.Several continuous vertebral bodies were destroyed.Thoracic vertebras were not involved.Mild expansive destruction of bone and vertebral accessories lesions were the characteristic of this disease.The surroundings of vertebra were the relatively obvious multilobulated juxtaarticular soft-tissue masses.The CT value of soft-tissue masses was usually lower than musculi parenchyma.The MRI signal of soft-tissue masses was heterogeneous and low signal on T1 WI,high signal on T2WI.Conclusion The CT and MRI imaging of chordoma are characteristics.Combined with the enhancement scanning,the site,range,morphology and the lesions of the surrounding tissue can be evaluated preferably.

  20. 臀大肌肌瓣在骶尾部脊索瘤手术治疗中的应用%The glutaeus maximus musculo flap in the treatment of sacrococcygeal chordoma

    Institute of Scientific and Technical Information of China (English)

    钟雷; 冯海龙


    探讨臀大肌肌瓣在骶尾部脊索瘤手术治疗中的应用.方法 1996年12月至2011年7月间,共有20例骶尾骨脊索瘤的患者在我院接受手术治疗.均采用后方入路广泛切除肿瘤,早期12例病例未采用臀大肌肌瓣,后期8例采用臀大肌肌瓣填塞缺损.结果 两组均有数例出现膀胱控尿功能及大便控制能力受损,但后期病例复发率和切口并发症发生率明显降低.结论 骶尾部脊索瘤的最佳治疗为广泛的手术切除,但术后容易出现切口并发症及外形不佳,采用臀大肌肌瓣填塞,可大大减少切口并发症的发生,效果满意.%Objective To investigate the glutaeus maximus musculo flap in the treatment of sacroco'ccygeal chordo-ma. Methods 20 cases of sacrococcygeal chordoma patients were accepted with operation treatment. All patients Using posterior approach with wide resection of the tumor, early 12 cases did not adopt the gluteus maximus musculo flap, later in 8 patients using the glutaeus maximus musculo flap packing defect. Results The two groups are few cases of bladder urination and bowel control ability is impaired, but late recurrence rate and incision complication rate was reduced significantly. Conclusion A wide range of operation excision is the best therapy for sacrococcygeal chordoma, but postoperative prone to wound complications and poor appearance, using the glutaeus maximus musculo flap packing, the incision complications can reduce greatly, The effectis satisfactory.

  1. Skull Base Chordoma in Children and Adolescents: Clinical Features and Management%儿童及青少年颅底脊索瘤的临床特征及治疗方案

    Institute of Scientific and Technical Information of China (English)

    栾世海; 孙琳琳; 鲍伟民; 杨伯捷; 陈宏; 毛颖; 曹晓运


    目的:阐述儿童及青少年颅底脊索瘤的临床特征及治疗方法,并分析影响其预后的因素.方法:对12例儿童及青少年颅底脊索瘤的临床表现、影像学特征、病理类型、手术及放射治疗等临床资料进行回顾性分析.结果:12例患者中,男性3例、女性9例,男女性别比为1:31发病年龄5~17岁,平均13.2岁.呕吐、头痛及复视为最常见的临床表现.病变多发于斜坡-后颅窝,多有斜坡骨质破坏.病理检查典型脊索瘤10例,软骨样脊索瘤2例.12例患者共行14次手术,首次手术5例全切,5例次全切,2例部分切除.随访6d至78个月(平均31.5个月),不同年龄、手术切除程度和是否接受放疗对患者的生存期有影响,差异有统计学意义(P<0.05).结论:在儿童及青少年颅底脊索瘤患者中,年龄较大者(10~18岁)具有较好的预后生存期,手术全切及放疗为相对有效的治疗方法.%Aim: To elucidate clinical features and management of skull base chordomas in children and adolescents, and to evaluate the factors affecting the long-term survivals. Methods: Clinical data of 12 children and adolescents with skull base chordomas, who were surgically treated in a single institution over a 20-year period, were retrospectively reviewed and analyzed. Results: There were 3 males and 9 females, aged from 5 to 17 years old, mean 13.2 years old. Vomiting, headache and diplopia were the most frequent symptoms or signs. Clivus and posterior cranial fossa were most involved, and bone destruction was noted usually. Conventional and chondroid chordomas comprised 83.3% and 16.7% of all cases, respectively. A total of 14 procedures were performed in patients. In the first procedure, 5 patients received total resection, 5 patients subtotal resection and 2 patients partial resection. Follow-up data were available for all the patients. The period ranged from 6 days to 78 months (mean 31.5 months). There were statistical significant

  2. CT and MRI findings of the chordoma in the mobile spine%脊柱活动节段脊索瘤的 CT、MRI 征象分析

    Institute of Scientific and Technical Information of China (English)

    庞超楠; 刘晓光; 袁慧书


    Objective To study the CT and MRI features of the chordoma in the mobile spine.Methods The CT and MRI fea-tures were retrospectively analyzed in twenty-four cases of chordoma in the mobile spine.Results In all the twenty-four cases,bone lesions were solitary in eight cases and multiple in sixteen cases,mostly occurred in cervical,occasionally occurred in thoracic or lum-bar vertebra.Both vertebral body and part appendix were involved in all cases.CT imaging showed that all the lesions mainly mani-fested as osteolytic bone destruction,peripheral osteosclerosis and the cortical bone were incomplete.Soft-tissue mass involved para-vertebral and intraspinal.Five lesions appeared vertebrae compression.Eight lesions appeared intervertebral foramen expansion.Le-sions appeared honeycomb or annular enhancement after contrast media injection.Lesions were presented as heterogeneous slightly hypo-isointensity on T1 WI,hyperintensity on T2 WI.Low signal fibrous septa within the tumors were seen.Lesions appeared hon-eycomb enhancement after Gd-DTPA injection.Conclusion CT and MRI findings of chordoma in the mobile spine can be regarded as characteristic,which are helpful for clinical diagnosis.%目的:探讨脊柱活动节段脊索瘤的 CT 和 MRI 表现。方法回顾分析24例经病理证实的脊柱活动节段脊索瘤的 CT、MRI 表现。结果24例病例中单发病变8例,多发病变16例,病变多发生在颈椎,也可发生在胸椎或腰椎;全部病例均同时累及椎体及部分附件结构。CT 显示全部病例均表现为不同程度的溶骨性骨质破坏,周围可见骨质硬化,骨皮质不完整,可见软组织肿块侵犯椎管内外;5例可见病变椎体压缩,8例可见椎间孔扩大;增强扫描病变呈蜂窝状或环形强化。MR 显示病变表现为不均匀等或稍长 T1长 T2信号,病灶内可见低信号间隔;增强扫描病变呈蜂窝状强化。结论脊柱活动节段脊索瘤的 CT、MRI 表现具有一定特征性,是临床诊断的重要依据。

  3. Trial of Dasatinib in Advanced Sarcomas (United States)


    Rhabdomyosarcoma; Malignant Peripheral Nerve Sheath Tumors; Chondrosarcoma; Sarcoma, Ewing's; Sarcoma, Alveolar Soft Part; Chordoma; Epithelioid Sarcoma; Giant Cell Tumor of Bone; Hemangiopericytoma; Gastrointestinal Stromal Tumor (GIST)

  4. 78 FR 16690 - Government-Owned Inventions; Availability for Licensing (United States)


    ... Treatment of Chordoma Description of Technology: Utilizing high-throughput screening methodology, NIH scientists have identified two classes of clinically- available drugs, proteasome inhibitors and... accidental radiation exposure. This discovery can also increase the therapeutic efficacy of...

  5. En bloc Resection of a Multilevel High-cervical Chordoma Involving C-2 and C-3 Using 3D Printing%3D打印技术辅助颈椎高位多节段脊索瘤手术临床应用

    Institute of Scientific and Technical Information of China (English)

    马立敏; 张余; 周烨; 陈加荣; 蓝国波; 黄文汉; 姚孟宇



  6. 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)


    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.)

  7. Evolution of Minimally Invasive Approaches to the Sella and Parasellar Region


    Louis,Robert G.; Eisenberg, Amy; Barkhoudarian, Garni; Griffiths, Chester; Kelly, Daniel F.


    Introduction Given advancements in endoscopic image quality, instrumentation, surgical navigation, skull base closure techniques, and anatomical understanding, the endonasal endoscopic approach has rapidly evolved into a widely utilized technique for removal of sellar and parasellar tumors. Although pituitary adenomas and Rathke cleft cysts constitute the majority of lesions removed via this route, craniopharyngiomas, clival chordomas, parasellar meningiomas, and other lesions are increasingl...

  8. [The combination treatment of malignant bone tumors using fast neutrons]. (United States)

    Chernichenko, V A; Tolstopiatov, B A; Konovalenko, V F; Monich, A Iu; Palivets, A Iu


    The study deals with results of a clinical trial evaluating treatment efficacy of a 6 MeV neutron beam produced by Y-120 cyclotron (Kiev). Procedures of preoperative radiotherapy and radical treatment are discussed. Radiotherapy was administered to 52 patients suffering chondrosarcoma (30 cases), osteogenic sarcoma (15) or chordoma (7). Combined treatment (radiation + surgery) was given to 22 patients whereas neutron beam therapy--to 30. All patients with osteogenic sarcoma received adjuvant combination chemotherapy. Three-year survival rate was compared to that observed in controls in whom combined treatment had included gamma-therapy. A significant increase in three-year survival rate was observed for osteogenic sarcoma and chordoma whereas for chondrosarcoma the improvement in survival proved insignificant. The use of fast neutrons in combined treatment of bone tumors was considered promising.

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

    Directory of Open Access Journals (Sweden)

    PAN Bin-cai


    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

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

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    Ernesto Mazza


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

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

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


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

  12. Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma (United States)


    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

  13. MicroRNA Gene Regulatory Networks in Peripheral Nerve Sheath Tumors (United States)


    De Vito C, Provero P, Stehle JC, Baumer K, Cironi L, Janiszewska M, Petricevic T, Suva D, Tercier S, Joseph JM, Guillou L, Stamenkovic I (2010) EWS...chordoma: c-Met oncoprotein. Spine 33(25):2774–2780. doi:10.1097/BRS.0b013e31817e2d1e 76. Felli N, Fontana L, Pelosi E, Botta R, Bonci D, Facchiano F

  14. Sellar tumors; Tumoren der Sellaregion

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    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


    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.)

  15. 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


    (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......) 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...

  16. [Treatment with charged particles beams: hadrontherapy part I: physical basis and clinical experience of treatment with protons]. (United States)

    Noël, G; Feuvret, L; Ferrand, R; Mazeron, J-J


    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 rarety and the cost of equipments.

  17. Augmented reality-assisted skull base surgery. (United States)

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


    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.

  18. MR findings of desmoplastic fibroma of the spine. A case report

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    Kong, K.Y.; Kang, H.S.; Jung, H.W.; Kim, J.J.; Lee, C.K. [Seoul National Univ. College of Medicine (Korea, Republic of). Dept. of Radiology


    We report on the MR imaging findings in a case of spinal desmoplastic fibroma (DF). DF of the bone is a rare, locally aggressive tumor. It is commonly located in long bones, pelvis or mandible. DF involving the spine is extremely rare and difficult to distinguish from other bony lesions such as giant cell tumor, chordoma and fibrous dysplasia of the spine. This case of DF of the spine showed MR findings similar to those of DF arising in the metaphysis of a long bone.

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

    Directory of Open Access Journals (Sweden)

    Faris Shweikeh


    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.

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

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


    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.)

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

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    Grosshans, David R., E-mail: [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)


    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

  2. Proposed Diagnostic Criteria, Classification Schema, and Review of Literature of Notochord-Derived Ecchordosis Physaliphora. (United States)

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


    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.

  3. 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)


    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.)

  4. 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


    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.

  5. Differential diagnosis of dumbbell lesions associated with spinal neural foraminal widening: Imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Kivrak, Ali Sami [Selcuk University, Meram Medical Faculty, Department of Radiology, 42080 Konya (Turkey)], E-mail:; Koc, Osman; Emlik, Dilek; Kiresi, Demet; Odev, Kemal [Selcuk University, Meram Medical Faculty, Department of Radiology, 42080 Konya (Turkey); Kalkan, Erdal [Selcuk University, Meram Medical Faculty, Department of Neurosurgery, Konya (Turkey)


    Computed tomography (CT) and magnetic resonance imaging (MRI) reliably demonstrate typical features of schwannomas or neurofibromas in the vast majority of dumbbell lesions responsible for neural foraminal widening. However, a large variety of unusual lesions which are causes of neural foraminal widening can also be encountered in the spinal neural foramen. Radiologic findings can be helpful in differential diagnosis of lesions of spinal neural foramen including neoplastic lesions such as benign/malign peripheral nerve sheath tumors (PNSTs), solitary bone plasmacytoma (SBP), chondroid chordoma, superior sulcus tumor, metastasis and non-neoplastic lesions such as infectious process (tuberculosis, hydatid cyst), aneurysmal bone cyst (ABC), synovial cyst, traumatic pseudomeningocele, arachnoid cyst, vertebral artery tortuosity. In this article, we discuss CT and MRI findings of dumbbell lesions which are causes of neural foraminal widening.

  6. MR imaging of the pelvis: a guide to incidental musculoskeletal findings for abdominal radiologists. (United States)

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


    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.

  7. 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)


    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.

  8. Midline and far lateral approaches to foramen magnum lesions.

    Directory of Open Access Journals (Sweden)

    Sharma B


    Full Text Available Twenty patients with foramen magnum lesions were operated upon in the last 5 years at Postgraduate Institute of Medical Education and Research, Chandigarh. The common presenting features were quadriparesis, quadriplegia, diminished sensations, neck pain and respiratory insufficiency. The lesions encountered were meningiomas, neurofibromas, posterior inferior cerebellar artery aneurysms, neurenteric cyst and chordoma. Patients with posterior or posterolaterally placed lesions were operated by the midline posterior approach while those with anterior or anterolateral lesions were managed by the far lateral approach. All mass lesions were excised completely and the aneurysms were clipped. Seventeen patients made good neurological recovery while three died. The latter three patients presented very late. The merits of various surgical approaches to the foramen magnum are discussed.

  9. 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))


    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.

  10. Primary bone tumors of the spine. (United States)

    Cañete, A Navas; Bloem, H L; Kroon, H M


    Primary bone tumors of the spine are less common than metastases or multiple myeloma. Based on the patient's age and the radiologic pattern and topography of the tumor, a very approximate differential diagnosis can be established for an osseous vertebral lesion. This article shows the radiologic manifestations of the principal primary bone tumors of the spine from a practical point of view, based on our personal experience and a review of the literature. If bone metastases, multiple myeloma, lymphomas, hemangiomas, and enostoses are excluded, only eight types of tumors account for 80% of all vertebral tumors. These are chordomas, osteoblastomas, chondrosarcomas, giant-cell tumors, osteoid osteomas, Ewing's sarcomas, osteosarcomas, and aneurysmal bone cysts.

  11. Osteochondroma of the sella turcica presenting with intratumoral hemorrhage. (United States)

    Inoue, Tomoo; Takahashi, Noboru; Murakami, Kensuke; Nishimura, Shinjitsu; Kaimori, Mitsuomi; Nishijima, Michiharu


    A 29-year-old man presented with a primary sellar turcica osteochondroma manifesting as intratumoral hemorrhage mimicking pituitary apoplexy. The patient suffered sudden onset of headache concomitant with vision loss in the left eye. Radiography and computed tomography detected destruction and calcification of the sellar turcica. Magnetic resonance imaging revealed a heterogeneously enhanced suprasellar mass that had elevated and compressed the optic chiasm. The preoperative diagnosis was hemorrhagic pituitary adenoma, craniopharyngioma, meningioma, or chordoma based on the signal heterogeneity of the lesion. To relieve the symptoms and make a definitive diagnosis, surgical removal via a basal interhemispheric approach was carried out. The tumor was not totally removed because of tight adhesion to the pituitary stalk, but postoperative ophthalmological examination revealed improvement of the visual disturbance. The histological diagnosis was osteochondroma based on the presence of mature chondrocytes and osteomatous tissue. Osteochondroma should be included in the differential diagnosis of tumors with acute hemorrhage in the sella turcica.

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

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


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

  13. Time-dependent change of dymanic MRI in brain and spinal tumors

    Energy Technology Data Exchange (ETDEWEB)

    Kikuchi, Yasuhiro; Matsumoto, Masato; Sato, Masanori; Fujita, Takashi; Suzuki, Kyoichi; Kodama, Namio (Fukushima Medical Coll. (Japan))


    Time-dependent changes of MR imaging were studied in 42 brain and 3 spinal tumors. Fourteen pituitary adenomas, 12 meningiomas, 7 neurinomas, 3 glioblastomas, 5 astrocytomas, 2 ependymomas, one chordoma, and one orbital pseudotumor were studied using the spin-echo technique (SE 600/20) after the injection of 0.1 mmol/kg of gadolinium-DTPA. The value of the signal intensities of the tumor tissue and the necrotic tissue were measured at pre- and postcontrast using a region-of-interest (ROI) technique. The signal intensity (S.I.) ratio of the contrast-enhanced area was calculated as follows: (postcontrast S.I.-precontrast S.I.) /precontrast S.I. x 100 (%). The time dependent curves of the S.I. ratio and the images were also evaluated. The time-S.I. ratio curves of the meningiomas, pituitary adenomas, neurinomas, ependymomas and pseudotumor showed a rapid increase, followed by a gradual decrease. The neurinomas were more enhanced than the other tumors. The pituitary adenomas showed a heterogeneous enhancement in delayed scans, but the meningiomas were sequentially homogeneous. The S.I. ratios of the astrocytomas (grade III), glioblastomas, and chordoma slowly increased until they reached a peak at from 5 to 20 min., and then decreased gradually. The initial enhanced areas in astrocytoma (grade III) and glioblastoma extended around sequentially, but the necrotic areas of the astrocytoma (grade III) and glioblastoma were gradually enhanced. The astrocytomas (grade II) showed no enhancement. Our study, using dynamic MR imaging, is useful because it gives more precise information for the differential diagnosis of brain tumors. (author).

  14. 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:; Ellerbrock, Malte; Haberer, Thomas (Heidelberger Ionenstrahl Therapiezentrum (HIT), Im Neuenheimer Feld 450, 69120 Heidelberg (Germany)) (and others)


    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.

  15. Endoscopic endonasal resection of skull base chordomas—classification and approachs%颅底脊索瘤的内镜经鼻手术治疗分型及入路

    Institute of Scientific and Technical Information of China (English)

    桂松柏; 宗绪毅; 王新生; 李储忠; 赵澎; 鲁润春; 张亚卓


    目的 探讨一种适合内镜经鼻手术治疗需要的颅底脊索瘤临床分型及入路选择方法.方法 回顾性分析2007年8月至2012年8月于我院使用内镜经鼻手术治疗的133例颅底脊索瘤病例资料.依据内镜经鼻手术斜坡解剖区域分类方法对脊索瘤进行临床分型.其中,主体位于颅底中线区域116例:(1)主体位于前颅底6例;(2)主体位于上斜坡7例;(3)主体位于上中斜坡42例;(4)主体位于中下斜坡8例;(5)主体位于下斜坡21例;(6)主体位于全斜坡32例.主体位于中线及中线旁区域(广泛型)17例.全部病人均行内镜经鼻手术切除.中线区域型共使用4种内镜经鼻手术入路:内镜经鼻-前颅底入路、内镜经鼻-上斜坡入路、内镜经鼻-中斜坡入路、内镜经鼻-下斜坡入路.广泛型使用内镜经鼻手术入路结合其他开颅手术入路进行肿瘤切除.结果 病变全切为26例(20%),次全切62例(47%),大部切除38例(29%),部分切除7例(5%).结论 制定适合内镜经鼻手术的斜坡解剖区域划分,并以此为基础对颅底脊索瘤进行临床分型,可以更好地指导内镜经鼻切除颅底脊索瘤的手术入路选择.%Objective To investigate a schematic anatomo-surgical classification suitable for Endoscopic endonasal resection of Skull base Chordomas,based on which to select selection of the appropriate Endoscopic endonasal surgical approach.Methods The records of 133 patients who presented with Skull base Chordoma between 2007.8 and 2012.8 were retrospectively reviewed.These lesions were classified according to their anatomical location as follows:(i) midline area:anterior skull base (6 cases),upper clivus (7 cases),upper-middle clivus (42 cases),middle-lower clivus (8 cases),lower clivus (21 cases),whole clivus (32cases);and (ii) midline and paramedian area:widespread (17 cases).The Endoscopic endonasal approaches were used for chordomas of midline area include anterior skull base

  16. Integrated and Quantitative Proteomics of Human Tumors. (United States)

    Yakkioui, Y; Temel, Y; Chevet, E; Negroni, L


    Quantitative proteomics represents a powerful approach for the comprehensive analysis of proteins expressed under defined conditions. These properties have been used to investigate the proteome of disease states, including cancer. It has become a major subject of studies to apply proteomics for biomarker and therapeutic target identification. In the last decades, technical advances in mass spectrometry have increased the capacity of protein identification and quantification. Moreover, the analysis of posttranslational modification (PTM), especially phosphorylation, has allowed large-scale identification of biological mechanisms. Even so, increasing evidence indicates that global protein quantification is often insufficient for the explanation of biology and has shown to pose challenges in identifying new and robust biomarkers. As a consequence, to improve the accuracy of the discoveries made using proteomics in human tumors, it is necessary to combine (i) robust and reproducible methods for sample preparation allowing statistical comparison, (ii) PTM analyses in addition to global proteomics for additional levels of knowledge, and (iii) use of bioinformatics for decrypting protein list. Herein, we present technical specificities for samples preparation involving isobaric tag labeling, TiO2-based phosphopeptides enrichment and hydrazyde-based glycopeptides purification as well as the key points for the quantitative analysis and interpretation of the protein lists. The method is based on our experience with tumors analysis derived from hepatocellular carcinoma, chondrosarcoma, human embryonic intervertebral disk, and chordoma experiments.

  17. Decisions and involvement of cancer patient survivors: a moral imperative

    Directory of Open Access Journals (Sweden)

    Pravettoni G


    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

  18. CT and MRI of intrinsic space-occupying lesions of the bony skull base; CT und MRT bei intrinsischen raumfordernden Laesionen der knoechernen Schaedelbasis

    Energy Technology Data Exchange (ETDEWEB)

    Koesling, S.; Brandt, S. [Martin-Luther-Universitaet Halle-Wittenberg, Universitaetsklinik und Poliklinik fuer Diagnostische Radiologie, Halle (Germany); Neumann, K. [Martin-Luther-Universitaet Halle-Wittenberg, Universitaetsklinik und Poliklinik fuer Hals-, Nasen-, Ohrenheilkunde, Kopf- und Halschirurgie, Halle (Germany)


    Intrinsic bony lesions of the skull base are diseases which arise within the bones forming the skull base. Mainly they are bone tumours and tumour-like lesions. With the exception of osteomas of the paranasal sinuses and exostoses of the external auditory canal, these lesions occur rarely. This article gives an overview of the appearance of the most common primary bony skull base masses in CT and MRI. From the authors' point of view these are fibrous dysplasia, chordomas, chondrosarcomas, Langerhans cell histiocytosis and multiple myelomas, which must be differentiated from pseudolesions. The possibilities of CT and MRI in making a specific diagnosis, differential diagnosis and the kind of making the final diagnosis are described. (orig.) [German] Unter intrinsischen Laesionen der knoechernen Schaedelbasis versteht man Erkrankungen, die von den die Schaedelbasis bildenden Knochen ausgehen. In erster Linie handelt es sich um Knochentumoren und tumoraehnliche Laesionen. Mit wenigen Ausnahmen (Nasennebenhoehlenosteome, Exostosen des aeusseren Gehoergangs) sind sie selten. Dieser Beitrag gibt einen Ueberblick ueber das CT- und MRT-Erscheinungsbild der aus Sicht der Autoren unter diesem Aspekt noch am haeufigsten anzutreffenden raumfordernden Laesionen. Dazu zaehlen die fibroese Dysplasie, Chordome, Chondrosarkome, die Langerhans-Zell-Histiozytose und das multiple Myelom. Abgrenzt werden muessen Pseudotumoren. Artdiagnostische Zuordnungsmoeglichkeiten durch CT und/oder MRT, Differenzialdiagnosen und Diagnosesicherung werden dargelegt. (orig.)

  19. Lesions in Meckel's cave: variable presentation and pathology. (United States)

    Beck, D W; Menezes, A H


    A series of 12 patients with mass lesions arising from Meckel's cave is presented. Patients' age on presentation ranged from 13 months to 71 years. Nine of the 12 patients had symptoms referable to the fifth cranial nerve, but only three complained of facial pain. The 12 patients presented eight different pathological entities, including meningioma, lipoma, schwannoma, malignant melanotic schwannoma, arachnoid cyst, neurofibroma, epidermoid tumor, and chordoma. Computerized tomography and magnetic resonance imaging were most useful in localizing the lesion to Meckel's cave. All 12 patients underwent a subtemporal approach to the lesion, and gross total removal was achieved in 11. Postoperative results were excellent with no increased neurological deficits seen 3 months postoperatively. Most patients had resolution of the cranial nerve deficits except for fifth nerve function, which was impaired in nine patients postoperatively. This series demonstrates that lesions in Meckel's cave can have a varied and unusual presentation, as well as an assortment of pathology. Total removal of lesions in this area resulted in relief of symptoms in most patients, with minimum morbidity.

  20. Particle beam therapy (hadrontherapy): basis for interest and clinical experience. (United States)

    Orecchia, R; Zurlo, A; Loasses, A; Krengli, M; Tosi, G; Zurrida, S; Zucali, P; Veronesi, U


    The particle or hadron beams deployed in radiotherapy (protons, neutrons and helium, carbon, oxygen and neon ions) have physical and radiobiological characteristics which differ from those of conventional radiotherapy beams (photons) and which offer a number of theoretical advantages over conventional radiotherapy. After briefly describing the properties of hadron beams in comparison to photons, this review discusses the indications for hadrontherapy and analyses accumulated experience on the use of this modality to treat mainly neoplastic lesions, as published by the relatively few hadrontherapy centres operating around the world. The analysis indicates that for selected patients and tumours (particularly uveal melanomas and base of skull/spinal chordomas and chondrosarcomas), hadrontherapy produces greater disease-free survival. The advantages of hadrontherapy are most promisingly realised when used in conjunction with modern patient positioning, radiation delivery and focusing techniques (e.g. on-line imaging, three-dimensional conformal radiotherapy) developed to improve the efficacy of photon therapy. Although the construction and running costs of hadrontherapy units are considerably greater than those of conventional facilities, a comprehensive analysis that considers all the costs, particularly those resulting from the failure of less effective conventional radiotherapy, might indicate that hadrontherapy could be cost effective. In conclusion, the growing interest in this form of treatment seems to be fully justified by the results obtained to date, although more efficacy and dosing studies are required.

  1. The expanding role of the endonasal endoscopic approach in pituitary and skull base surgery: A 2014 perspective (United States)

    Lobo, Bjorn; Heng, Annie; Barkhoudarian, Garni; Griffiths, Chester F.; Kelly, Daniel F.


    Background: The past two decades have been the setting for remarkable advancement in endonasal endoscopic neurosurgery. Refinements in camera definition, surgical instrumentation, navigation, and surgical technique, including the dual surgeon team, have facilitated purely endonasal endoscopic approaches to the majority of the midline skull base that were previously difficult to access through the transsphenoidal microscopic approach. Methods: This review article looks at many of the articles from 2011 to 2014 citing endonasal endoscopic surgery with regard to approaches and reconstructive techniques, pathologies treated and outcomes, and new technologies under consideration. Results: Refinements in approach and closure techniques have reduced the risk of cerebrospinal fluid leak and infection. This has allowed surgeons to more aggressively treat a variety of pathologies. Four main pathologies with outcomes after treatment were identified for discussion: pituitary adenomas, craniopharyngiomas, anterior skull base meningiomas, and chordomas. Within all four of these tumor types, articles have demonstrated the efficacy, and in certain cases, the advantages over more traditional microscope-based techniques, of the endonasal endoscopic technique. Conclusions: The endonasal endoscopic approach is a necessary tool in the modern skull base surgeon's armamentarium. Its efficacy for treatment of a wide variety of skull base pathologies has been repeatedly demonstrated. In the experienced surgeon's hands, this technique may offer the advantage of greater tumor removal with reduced overall complications over traditional craniotomies for select tumor pathologies centered near the midline skull base. PMID:26015870

  2. Improving the endoscopic endonasal transclival approach: the importance of a precise layer by layer reconstruction. (United States)

    Iacoangeli, Maurizio; Di Rienzo, Alessandro; di Somma, Lucia Giovanna Maria; Moriconi, Elisa; Alvaro, Lorenzo; Re, Massimo; Salvinelli, Fabrizio; Carassiti, Massimiliano; Scerrati, Massimo


    BACKGROUND. The endoscopic endonasal transclival approach (EETCA) is a minimally-invasive technique allowing a direct route to the base of implant of clival lesions with reduced brain and neurovascular manipulation. On the other hand, it is associated with potentially severe complications related to the difficulties in reconstructing large skull base defects with a high risk of postoperative cerebrospinal fluid (CSF) leakage. The aim of this paper is to describe a precise layer by layer reconstruction in the EETCA including the suture of the mucosa as an additional reinforcing layer between cranial and nasal cavity in order to speed up the healing process and reduce the incidence of CSF leak. METHODS. This closure technique was applied to the last six cases of EETCA used for clival meningiomas (2), clival chordomas (2), clival metastasis (1), and craniopharyngioma with clival extension (1). RESULTS. After a mean follow-up of 6 months we had no one case of postoperative CSF leakage or infections. Seriated outpatient endoscopic endonasal controls showed a fast healing process of nasopharyngeal mucosa with less patient discomfort. CONCLUSIONS. Our preliminary experience confirms the importance of a precise reconstruction of all anatomical layers violated during the surgical approach, including the nasopharygeal mucosa.

  3. Chondromyxoid fibroma of the sacrum: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Tanya Minasian


    Full Text Available Background: Chondromyxoid fibroma (CMF is an extremely rare, benign cartilaginous tumor that makes up <0.5% of all bone tumors, typically presenting in the second or third decade of life. CMF of the sacrum is exceedingly rare, with only seven documented cases reported in the neurosurgical literature. Case Description: We report a case of a 35-year-old female with a 3 month history of lower back pain after sustaining a fall on her sacrum/coccyx presenting with a progressive complaint of localized lower back pain, occasional urinary retention without incontinence, gluteal hypesthesia, and pressure below the gluteal crease. Imaging demonstrated a large, expansile enhancing soft-tissue lesion involving the sacrum, distal to the S2-3 disc space. The tumor was removed with partial sacrectomy for open en bloc resection with partial nerve sparing. The patient was found at 1.5-year follow-up with the improvement of symptoms, no recurrence, and no residual neurologic dysfunction. Conclusion: Sacral CMF is a rare clinical entity that may mirror more aggressive sacral pathology, including chordoma, in both clinical presentation and imaging characteristics. A review of the available literature regarding diagnosis, surgical management options, and prognosis for sacral CMF is provided.

  4. Proton Radiation Therapy for Head and Neck Cancer: A Review of the Clinical Experience to Date

    Energy Technology Data Exchange (ETDEWEB)

    Holliday, Emma B.; Frank, Steven J., E-mail:


    Proton beam radiation has been used for cancer treatment since the 1950s, but recent increasing interest in this form of therapy and the construction of hospital-based and clinic-based facilities for its delivery have greatly increased both the number of patients and the variety of tumors being treated with proton therapy. The mass of proton particles and their unique physical properties (ie, the Bragg peak) allow proton therapy to spare normal tissues distal to the tumor target from incidental irradiation. Initial observations show that proton therapy is particularly useful for treating tumors in challenging locations close to nontarget critical structures. Specifically, improvements in local control outcomes for patients with chordoma, chonodrosarcoma, and tumors in the sinonasal regions have been reported in series using proton. Improved local control and survival outcomes for patients with cancer of the head and neck region have also been seen with the advent of improvements in better imaging and multimodality therapy comprising surgery, radiation therapy, and chemotherapy. However, aggressive local therapy in the proximity of critical normal structures to tumors in the head and neck region may produce debilitating early and late toxic effects. Great interest has been expressed in evaluating whether proton therapy can improve outcomes, especially early and late toxicity, when used in the treatment of head and neck malignancies. This review summarizes the progress made to date in addressing this question.

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

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Tae Ho; Lee, Ho Suk; Kim, Yong Joo; Kim, Tae Hun; Suh, Kyung Jin; Kang, Duk Sik [College of Medicine, Kyungpook National University, Daegu (Korea, Republic of)


    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.

  6. Gastrointestinal stromal tumor and other primary metachronous or synchronous neoplasms as a suspicion criterion for syndromic setting. (United States)

    Ponti, Giovanni; Luppi, Gabriele; Martorana, Davide; Rossi, Giulio; Losi, Lorena; Bertolini, Federica; Sartori, Giuliana; Pellacani, Giovanni; Seidenari, Stefania; Boni, Elisa; Neri, Tauro Maria; Silini, Enrico; Tamburini, Elisa; Maiorana, Antonio; Conte, Pier Franco


    Gastrointestinal stromal tumors (GISTs) may be sporadic or inherited. Although KIT and PDGFRA activating mutations are the oncogenic mechanisms in most sporadic and inherited GISTs, a small subset of GISTs are negative for both. Besides the classical Familial GIST Syndrome, GIST can occur as part of multi-neoplastic disease. The present study was designed to analyze the synchronous and metachronous tumors developed among GIST patients assessed by our institution for GIST Syndrome setting recognition. Patients (n=141) with primary GIST (77 men and 64 women) were recruited between 1988 and 2007 and their clinical and pathological records were reviewed. Mutation analysis of KIT, PDGFRA, NF1 and MMR genes was performed on somatic and peripheral blood DNA. GISTs occurred associated with other primary malignancies in 46 of 141 (32.6%) patients. The most common neoplasms were gastrointestinal and genitourinary. A novel exon 6 germline large deletion of NF1 was identified in the NF1/GIST kindred. The development of GIST associated with other neoplasms is common and diagnosis of peculiar benign associated-neoplasms warrants the search for familial cancer susceptibility. In particular, syndromic or familial settings have to be suspected in the presence of neurofibroma or lung chordoma in C-KIT and PDGFRA negative GIST patients.

  7. The utility of 3D printing for surgical planning and patient-specific implant design for complex spinal pathologies: case report. (United States)

    Mobbs, Ralph J; Coughlan, Marc; Thompson, Robert; Sutterlin, Chester E; Phan, Kevin


    OBJECTIVE There has been a recent renewed interest in the use and potential applications of 3D printing in the assistance of surgical planning and the development of personalized prostheses. There have been few reports on the use of 3D printing for implants designed to be used in complex spinal surgery. METHODS The authors report 2 cases in which 3D printing was used for surgical planning as a preoperative mold, and for a custom-designed titanium prosthesis: one patient with a C-1/C-2 chordoma who underwent tumor resection and vertebral reconstruction, and another patient with a custom-designed titanium anterior fusion cage for an unusual congenital spinal deformity. RESULTS In both presented cases, the custom-designed and custom-built implants were easily slotted into position, which facilitated the surgery and shortened the procedure time, avoiding further complex reconstruction such as harvesting rib or fibular grafts and fashioning these grafts intraoperatively to fit the defect. Radiological follow-up for both cases demonstrated successful fusion at 9 and 12 months, respectively. CONCLUSIONS These cases demonstrate the feasibility of the use of 3D modeling and printing to develop personalized prostheses and can ease the difficulty of complex spinal surgery. Possible future directions of research include the combination of 3D-printed implants and biologics, as well as the development of bioceramic composites and custom implants for load-bearing purposes.

  8. 浅谈骨肿瘤诊治中的底线%Bottom line in the treatment of bone tumors

    Institute of Scientific and Technical Information of China (English)



    Medicine is the science of human diseases and health. The principles of all the treatment methods and techniques should be in consistent with what has been recognized in the treatment of bone and soft tumors. Clinical Practice Guidelines for Bone Cancer ( NCCN Guidelines ) printed by National Comprehensive Cancer Network is well known, in which the treatment of chondrosarcoma, chordoma, ewing’s sarcoma, giant cell tumor of bone and osteosarcoma were discussed in detail. Most important, principles of bone cancer management were emphasized in this guideline, including: ( 1 ) Biopsy diagnosis is necessary prior to any surgical procedures. ( 2 ) Wide excision should achieve histologically negative surgical margins. ( 3 ) Lab studies such as CBC, LDH, and ALP may have relevance in the diagnosis. ( 4 ) Care for bone cancer patients should be delivered directly by physicians of a multidisciplinary team. ( 5 ) Patients should have a survivorship prescription to schedule follow-up with a multidisciplinary team. Respect on the spirits of science is the bottom line of medicine, and we should regard the principle and guideline mentioned above as the bottom line in the treatment of bone and soft tumors.


    Institute of Scientific and Technical Information of China (English)

    Jian Yin; Chang-bao Su; Zhi-qin Xu; Xue-wei Xia; Fei Song


    Objective To introduce a new principle of sellar reconstruction and to evaluate the effectiveness of absorbable gelatin foam and fibrin glue for sellar reconstruction. Methods A total of 176 consecutive patients who underwent surgery for pituitary adenomas, cysts, chordomas, or subdiaphragmatic craniopharyngiomas in the sella turcica between January 2001 and April 2003 at Peking Union Medical College Hospital were enrolled. Different techniques of sellar closure and indications for each specific condition were retrospectively reviewed. Results Seventy-seven (43.7%) patients developed a visible cerebrospinal fluid (CSF) leakage during surgery. Intra operative CSF leakage were repaired simply with gelatin foam and fibrin glue in 62 (35.2%) patients, and with autologous fat graft and sellar floor reconstruction in 15 (8.5%) patients. Postoperative CSF rhinorrhea occurred only in 1 case. There were no visual deterioration, allergic rhinitis, meningitis, pneumocranium, granulomas, or other complications associated with the reconstruction procedure. Conclusion The procedure of using gelatin foam and fibrin glue and principle of cranial base reconstruction is safe and effective in preventing postoperative complications following transsphenoidal surgery.

  10. Sellar Chondroma with Endocrine Dysfunction that Resolved after Surgery: Case Report. (United States)

    Hattori, Yujiro; Tahara, Shigeyuki; Nakakuki, Takuya; Takei, Mao; Ishii, Yudo; Teramoto, Akira; Morita, Akio


    Chondromas originating from the sella turcica are rare, and the most common initial symptoms are headache and visual disturbance. We describe a case of sellar chondroma with endocrine impairment as an initial manifestation that completely resolved after surgery. A 40-year-old Japanese woman with amenorrhea and galactorrhea for the last 2 years was referred to our department of neurosurgery for the evaluation of high prolactin levels and a tumor in the sella turcica. A biochemical assessment indicated endocrine dysfunction. Magnetic resonance imaging and computed tomography indicated a tumor in the sella turcica. The patient's presentation favored the preoperative diagnosis of pituitary adenoma or Rathke's cleft cyst. However, because calcification was detected, other types of tumors, such as craniopharyngioma, meningioma, and chordoma, were also considered. Endoscopic transsphenoidal surgery was performed, and the possibility of a bony tumor was recognized. Finally, the tumor was completely removed, and the histopathological findings confirmed chondroma. The postoperative course was uneventful, and endocrine function improved. Five years after surgery, the patient is doing well without pituitary insufficiency, pituitary hormone medications, or signs of tumor recurrence. In cases of sellar chondroma, endocrine dysfunction sometimes precedes other symptoms, such as headache and visual disturbance. When examining a patient with an intrasellar tumor harboring calcification, clinicians must consider the possibility of sellar chondroma. Furthermore, to the best of our knowledge, this case is the first of sellar chondroma treated with endoscopic surgery to be reported.

  11. [Intracranial chondroma arising from the skull base: two case reports featuring the image findings for differential diagnosis]. (United States)

    Higashida, Tetsuhiro; Sakata, Katsumi; Kanno, Hiroshi; Tanabe, Yutaka; Kawasaki, Takashi; Yamamoto, Isao


    We reported two cases of intracranial skull base chondroma and discussed the differential diagnosis and the treatment strategies. The first case was a 39-year-old male who presented with left exophtalmos, visual loss and oculomotor disturbance. MRI showed a huge tumor occupying the bilateral cavernous sinus. Partial removal of the tumor was performed through the left orbitozygomatic subtemporal approach. The second case was a 54-year-old male who presented with left hemiparesis. MRI showed a brain stem infarction with a huge tumor located at the right middle fossa. Partial removal was performed through the right orbitozygomatic subtemporal approach. In these two cases, the histopathological diagnosis of the tumors was benign chondroma and the size of residual tumors have not changed for one year without any additional therapy. Although preoperative definite diagnosis for skull base chondromas is difficult, strategies for diagnosis and treatment without any complication are essential. In our cases, chondromas showed low uptake in PET images, which might be useful for differentiation between chondromas and chordomas. The current popular surgical approach for parasellar tumors is transcranial such as the orbitozygomatic subtemporal approach. In surgical removal of skull base chondromas, it is advisable to try to confirm the diagnosis preoperatively with characteristic image findings and to consider the best approach in each case to decompress the involved nerves without any complications.

  12. 经耳前颧弓硬膜外入路切除海绵窦肿瘤60例分析%Resection of cavernous sinus tumor with subtomperal preauricular extradural approach:clinical experience and analysis of 60 cases

    Institute of Scientific and Technical Information of China (English)

    卜博; 周定标; 余新光; 许百男; 张远征; 乔广宇; 周涛


    目的 总结回顾2002年至2009年经治的海绵窦病变60例的临床特点、手术人路及手术效果.方法 神经鞘瘤18例,海绵状血管瘤23例,皮样囊肿9例,脑膜瘤4例,脊索瘤3例,垂体瘤3例.均经耳前颧弓硬膜外入路切除.结果 神经鞘瘤18均全切,海绵状血管瘤23例,全切18例,5例有残留.皮样囊肿9例全切,脑膜瘤4例,全切3例,次全切1例.脊索瘤3例,结合经鼻蝶窦入路手术,均达到了全切.垂体瘤3例全切.结论 经耳前颧弓硬膜外入路切除海绵窦病变是一个理想的手术入路,可以充分显露病变,减少对脑组织的牵拉,也可以明确Ⅲ~Ⅵ脑神经和颈内动脉的位置,减少神经和血管损伤的概率.对与动脉或神经粘连无法彻底切除的病变可以辅以立体定向放射治疗.%Objective To review our experience of microsurgery for 60 cavernous sinus tumors from 2002 to 2009.The clinical features,surgical techniques and outcome of cavernous sinus tumor in 60 cases were investigated retrospectively.Methods The patients included 23 hemangiomas,18 shwannomas,9 dermoid cysts,4 meningiomas,3 chordomas,3 pituitary adenomas.AIl the tumors were removed with subtomperal preauricular extradural approach.Results The tumors were removed satisfactorily.The shwannomas were totally removed. The hemangiomas were totally removed in 18,near-totally removed in 5 cases.Nine dermoid cysts were removed totally.For the 4 meningiomas,3 were removed completely,neartotallv removed in 1 cases.The 3 Chordomas were resected near-totally and achieved a completely removal with combined approach.Conclusion The subtomperal preauricular extradural approach is a rational choice.It can reveal the cranial nerve branches and artery at an early stage so that cranial nerves Ⅲ~Ⅵ and internal carotid artery can be preserved during operation.The tumor exposure is ideal and brain traction and contusion are slightly.The adjunctive radiotherapy is demanded for residual tumors

  13. 少见椎管内硬膜外占位性病变的MRI 表现%MR findings of rare spinal epidural mass

    Institute of Scientific and Technical Information of China (English)

    侯刚; 陈玉林; 夏钰弘; 汤群锋


    Objective To evaluate MR features of rare spinal epidural mass. Methods Seven patients with rare spinal epidural masses confirmed by pathologic examination were retrospectively analyzed. Results Spinal epidural abscess presented as a multilocular mass with low to intermediate signal intensity on T1WI, high signal intensity on T1WI and hypointense septa. Vertebral chordoma showed characteristic homogeneous slightly low T1 and high T1 signal intensities with honeycomb sign on T2WI. Chordoma of non -osseous origin presented as elliptical spinal epidural mass with homogeneous slightly low Tland high T2 signal intensities. Intraspinal granulocytic sarcoma presented as contrast - enhancing long stripes of low to intermediate T1 and heterogeneously high T2 signal intensities intermixed with epidural fat. Hemangiopericytoma was seen as an irregular branching spinal epidural mass with T1 and T2 isointensities and homogeneous contrast enhancement. Cystic ganglioneuroma presented as a grape-like multicystic mass with homogeneous low T1 and high T2 signal intensities as well as slightly hypointense septa. Conclusion MRI has value in demonstrating the epidural location of the masses and is helpful in the differential diagnosis of rare diseases.%目的 分析少见椎管硬膜外占位病变的MRI 特征.方法 对7 例病理及临床证实的椎管硬膜外占位的MR 表现进行回顾性分析.结果 硬膜外脓肿表现为多房样占位,形态较规则,T1WI 等、低混杂信号,T2WI 病灶呈高信号,房间隔低信号.骨源性脊索瘤表现为T1WI 均匀稍低信号,T2WI 高信号其内夹杂小点及小条状低信号呈蜂窝状改变.具有特征性影像表现.非骨源性脊索瘤表现为硬膜外长梭形占位,信号均匀,T1WI 稍低信号,T2WI 高信号,增强后病灶轻微强化.椎管内白血病浸润表现为硬膜外大范围长条状异常信号与硬膜外脂肪组织交杂存在,T1WI 呈等低混杂信号,T2WI 呈混杂高信号,增强后明显强

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


    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

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


    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

  16. Imaging appearances and clinical outcome following sacrectomy and ilio-lumbar reconstruction for sacral neoplasia

    Energy Technology Data Exchange (ETDEWEB)

    Thomas, Marianna; Davies, A.M.; James, Steven L.J. [Department of Radiology, The Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham (United Kingdom); Stirling, A.J.; Grainger, M. [Department of Spinal Surgery, The Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham (United Kingdom); Grimer, R.J. [Department of Orthopaedic Oncology, The Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham (United Kingdom)


    Sacrectomy and ilio-lumbar reconstruction is an uncommonly performed complex surgical procedure for the treatment of sacral neoplasia. There are many challenges in the post-operative period including the potential for tumor recurrence, infection, and construct failure. We present our experience of this patient cohort and describe the complications and imaging appearances that can be encountered during the follow-up period. Retrospective review of our Orthopaedic Oncology database was undertaken which has been collected over a 30-year period to identify patients that had undergone sacrectomy and ilio-lumbar reconstruction. Pre and post-operative imaging including radiographs, CT, and MRI was reviewed. These were viewed by two experienced musculoskeletal radiologists with consensus opinion if there was disagreement over the imaging findings. Data regarding patient demographics, tumor type, and dimensions was collected. Serial review of radiographs, CT, and MRI was performed to assess implant position and integrity, strut graft position and union, and for the presence of recurrence within the surgical bed. Five male and two female patients (mean age 36 years, age range 15-54 years) were treated with this procedure. Histological diagnoses included chordoma, chondrosarcoma, osteosarcoma, and spindle cell sarcoma. Mean maximal tumor size on pre-operative imaging was 10.7 cm (range, 6-16 cm). Post-operative follow-up ranged from 10-46 months. A total of 76 imaging studies were reviewed. Commonly identified complications included vertical rod and cross-connector fracture and screw loosening. Fibula strut graft non-union and fracture was also evident on imaging review. Two patients demonstrated disease recurrence during the follow-up period. This study demonstrates the spectrum and frequency of complications that can occur following sacrectomy and ilio-lumbar reconstruction for sacral neoplasia. (orig.)


    Directory of Open Access Journals (Sweden)

    E. S. Gershtein


    Full Text Available RANK/RANKL/OPG ligand-receptor system is a key player in bone homeostasis regulation directly regulating osteoclast differentiation and osteolysis. Disbalance of bone homeostasis associated with malfunctioning of RANK/RANKL/OPG system underlies such oncological processes as the destruction of bone, metastasis development, tumor progression. Involvement of RANK/RANKL/OPG system in the development of metastasis from various tumors is practically confirmed, but its influence on the development and progression of primary bone tumors still needs thorough evaluation. , In this paper experimental and clinical-laboratory data on the role of RANK/RANKL/OPG system in primary bone tumors pathogenesis available in modern literature are summarized with special attention,paid to giant-cell bone tumor (GCBT that is already treated with RANK/RANKL interaction inhibitor denosumab. Results of authors study of the levels of RANK/RANKL/OPG system,s components in blood serum of 101 malignant bone tumor (37 – osteosarcoma, 41 – chondrosarcoma, 12 – chordoma, 7 – Ewing sarcoma, 2 – pleomorhic undifferentiated sarcoma, 2 – fibrosarcoma, 32 borderline GCBT, and 30 benign bone tumor patients are also presented. The disturbances in the balance of osteolysis activators and inhibitors in patients with primary bone tumor depending both on the character of neoplasm (malignant, borderline or benign, and histological structure of malignant tumors were demonstrated. The most striking changes in RANK/ RANKL/OPG system manifesting itself in an increase of serum concentrations of all its components and strengthening of association between the levels of soluble receptor and its natural inhibitor OPG were revealed in giant-cell bone tumor patients.The study of the role of RANK/RANKL/OPG system in primary bone neoplasms is a topical goal for clinical investigations; it is also a promising tool for development of new diagnostic methods and for targeted application of

  18. Endoscopic endonasal skull base approach for parasellar lesions: Initial experiences, results, efficacy, and complications

    Directory of Open Access Journals (Sweden)

    Shigetoshi Yano


    Full Text Available Background: Endoscopic surgery is suitable for the transsphenoidal approach; it is minimally invasive and provides a well-lit operative field. The endoscopic skull base approach through the large opening of the sphenoid sinus through both nostrils has extended the surgical indication for various skull base lesions. In this study, we describe the efficacy and complications associated with the endoscopic skull base approach for extra- or intradural parasellar lesions based on our experiences. Methods: Seventy-four cases were treated by an endoscopic skull base approach. The indications for these procedures included 55 anterior extended approaches, 10 clival approaches, and 9 cavernous approaches. The operations were performed through both the nostrils using a rigid endoscope. After tumor removal, the skull base was reconstructed by a multilayered method using a polyglactin acid (PGA sheet. Results: Gross total resection was achieved in 82% of pituitary adenomas, 68.8% of meningiomas, and 60% of craniopharyngiomas in anterior extended approach and in 83.3% of chordomas in clival approach, but only in 50% of the tumors in cavernous approach. Tumor consistency, adhesion, and/or extension were significant limitations. Visual function improvements were achieved in 37 of 41 (90.2% cases. Cerebrospinal fluid (CSF leakage (9.5%, infections (5.4%, neural injuries (4.1%, and vascular injuries (2.7% were the major complications. Conclusions: Our experiences show that the endoscopic skull base approach is a safe and effective procedure for various parasellar lesions. Selection of patients who are unlikely to develop complications seems to be an important factor for procedure efficacy and good outcome.

  19. 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.


    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.

  20. Nivolumab and Ipilimumab in Treating Patients With Rare Tumors (United States)


    Acinar Cell Carcinoma; Adrenal Cortex Carcinoma; Adrenal Gland Pheochromocytoma; Anal Canal Neuroendocrine Carcinoma; Anal Canal Undifferentiated Carcinoma; Appendix Mucinous Adenocarcinoma; Bladder Adenocarcinoma; Bronchioloalveolar Carcinoma; Cervical Adenocarcinoma; Cervical Squamous Cell Carcinoma, Not Otherwise Specified; Cholangiocarcinoma; Chordoma; Colorectal Squamous Cell Carcinoma; Endometrial Adenocarcinoma; Endometrioid Adenocarcinoma; Esophageal Neuroendocrine Carcinoma; Esophageal Undifferentiated Carcinoma; Extrahepatic Bile Duct Carcinoma; Fallopian Tube Adenocarcinoma; Fibromyxoid Tumor; Gastric Neuroendocrine Carcinoma; Gastric Squamous Cell Carcinoma; Giant Cell Carcinoma; Intestinal Neuroendocrine Carcinoma; Intrahepatic Cholangiocarcinoma; Lung Carcinoid Tumor; Lung Sarcomatoid Carcinoma; Major Salivary Gland Carcinoma; Malignant Odontogenic Neoplasm; Malignant Peripheral Nerve Sheath Tumor; Malignant Skin Neoplasm; Malignant Testicular Sex Cord-Stromal Tumor; Metastatic Malignant Neoplasm of Unknown Primary Origin; Mixed Mesodermal (Mullerian) Tumor; Mucinous Adenocarcinoma; Mucinous Cystadenocarcinoma; Nasal Cavity Adenocarcinoma; Nasal Cavity Carcinoma; Nasopharyngeal Carcinoma; Nasopharyngeal Papillary Adenocarcinoma; Nasopharyngeal Undifferentiated Carcinoma; Oral Cavity Carcinoma; Oropharyngeal Undifferentiated Carcinoma; Ovarian Adenocarcinoma; Ovarian Germ Cell Tumor; Ovarian Mucinous Adenocarcinoma; Ovarian Squamous Cell Carcinoma; Pancreatic Acinar Cell Carcinoma; Pancreatic Neuroendocrine Carcinoma; Paraganglioma; Paranasal Sinus Adenocarcinoma; Paranasal Sinus Carcinoma; Parathyroid Gland Carcinoma; Pituitary Gland Carcinoma; Placental Choriocarcinoma; Placental-Site Gestational Trophoblastic Tumor; Primary Peritoneal High Grade Serous Adenocarcinoma; Pseudomyxoma Peritonei; Scrotal Squamous Cell Carcinoma; Seminal Vesicle Adenocarcinoma; Seminoma; Serous Cystadenocarcinoma; Small Intestinal Adenocarcinoma; Small Intestinal Squamous

  1. The endonasal microscopic approach for pituitary adenomas and other parasellar tumors: a 10-year experience. (United States)

    Fatemi, Nasrin; Dusick, Joshua R; de Paiva Neto, Manoel A; Kelly, Daniel F


    THE DIRECT ENDONASAL transsphenoidal approach to the sella with the operating microscope was initially described more than 20 years ago. Herein, we describe the technique, its evolution, and lessons learned over a 10-year period for treating pituitary adenomas and other parasellar pathology. From July 1998 to January 2008, 812 patients underwent a total of 881 operations for a pituitary adenoma (n = 605), Rathke's cleft cyst (n = 59), craniopharyngioma (n = 26), parasellar meningioma (n = 23), chordoma (n = 18), or other pathological condition (n = 81). Of these, 118 operations (13%) included an extended approach to the suprasellar, infrasellar/clival, or cavernous sinus regions. Endoscopic assistance was used in 163 cases (19%) overall, including 36% of the last 200 cases in the series and 18 (72%) of the last 25 extended endonasal cases. Surgical complications included 19 postoperative cerebrospinal fluid leaks (2%), 6 postoperative hematomas (0.7%), 4 carotid artery injuries (0.4%), 4 new permanent neurological deficits (0.4%), 3 cases of bacterial meningitis (0.3%), and 2 deaths (0.2%). The overall complication rate was higher in the first 500 cases in the series and in extended approach cases. Major technical modifications over the 10-year period included increased use of shorter (60-70 mm) endonasal speculums for greater instrument maneuverability and visualization, the micro-Doppler probe for cavernous carotid artery localization, endoscopy for more panoramic visualization, and a graded cerebrospinal fluid leak repair protocol. These changes appear to have collectively and incrementally made the approach safer and more effective. In summary, the endonasal approach provides a minimally invasive route for removal of pituitary adenomas and other parasellar tumors.

  2. Treatment of pediatric patients and young adults with particle therapy at the Heidelberg Ion Therapy Center (HIT: establishment of workflow and initial clinical data

    Directory of Open Access Journals (Sweden)

    Combs Stephanie E


    Full Text Available Abstract Background To report on establishment of workflow and clinical results of particle therapy at the Heidelberg Ion Therapy Center. Materials and methods We treated 36 pediatric patients (aged 21 or younger with particle therapy at HIT. Median age was 12 years (range 2-21 years, five patients (14% were younger than 5 years of age. Indications included pilocytic astrocytoma, parameningeal and orbital rhabdomyosarcoma, skull base and cervical chordoma, osteosarcoma and adenoid-cystic carcinoma (ACC, as well as one patient with an angiofibroma of the nasopharynx. For the treatment of small children, an anesthesia unit at HIT was established in cooperation with the Department of Anesthesiology. Results Treatment concepts depended on tumor type, staging, age of the patient, as well as availability of specific study protocols. In all patients, particle radiotherapy was well tolerated and no interruptions due to toxicity had to be undertaken. During follow-up, only mild toxicites were observed. Only one patient died of tumor progression: Carbon ion radiotherapy was performed as an individual treatment approach in a child with a skull base recurrence of the previously irradiated rhabdomyosarcoma. Besides this patient, tumor recurrence was observed in two additional patients. Conclusion Clinical protocols have been generated to evaluate the real potential of particle therapy, also with respect to carbon ions in distinct pediatric patient populations. The strong cooperation between the pediatric department and the department of radiation oncology enable an interdisciplinary treatment and stream-lined workflow and acceptance of the treatment for the patients and their parents.

  3. Skull base tumors; Tumoren der Schaedelbasis

    Energy Technology Data Exchange (ETDEWEB)

    Ahlhelm, F.; Naumann, N.; Grunwald, I.; Reith, W. [Klinik fuer Diagnostische und Interventionelle Neuroradiologie des Universitaetsklinikums des Saarlandes, Homburg/Saar (Germany); Nabhan, A.; Shariat, K. [Neurochirurgische Klinik des Universitaetsklinikums des Saarlandes, Homburg/Saar (Germany)


    Modern imaging techniques have great importance in the diagnosis and therapy of skull-base pathologies. Many of these lesions, especially in relation to their specific location, can be evaluated using CT and MR imaging. Tumors commonly found in the anterior skull base include carcinoma, rhabdomyosarcoma, esthesioneuroblastoma and meningioma. In the central cranial fossa, nasopharyngeal carcinoma, metastases, meningioma, pituitary adenoma and neurinoma have to be considered. The most common neoplasms of the posterior skull base, including the CP angle, are neurinoma, meningioma, nasopharyngeal carcinoma, chordoma and paraganglioma. One major task of imaging is the evaluation of the exact tumor extent as well as its relationship to the neighboring neurovascular structures. The purpose of this review is to recapitulate the most important anatomical landmarks of the skull base. The typical imaging findings of the most common tumors involving the skull base are also presented. (orig.) [German] Die moderne Bildgebung hat einen besonderen Stellenwert bei der Diagnostik und Therapie von Schaedelbasispathologien. Zahlreiche Laesionen koennen anhand ihrer CT- und MRT-Befunde, insbesondere unter Beruecksichtigung ihrer genauen Lokalisation, artdiagnostisch eingeordnet werden. Im Bereich der vorderen Schaedelbasis sind v. a. Karzinome, Rhabdomyosarkome, Aesthesioneuroblastome und Meningeome vorzufinden. Im Bereich der mittleren Schaedelbasis ist in erster Linie an nasopharyngeale Karzinome, Karzinommetastasen, Meningeome, Hypophysenadenome und Neurinome zu denken. Zu den haeufigsten Tumoren der hinteren Schaedelgrube, unter Einschluss des Kleinhirnbrueckenwinkels, gehoeren Neurinome, Meningeome, nasopharyngeale Karzinome, Karzinommetastasen, Chordome und Paragangliome. Eine wichtige Aufgabe der Schnittbildgebung liegt in der Bestimmung der exakten Tumorausdehnung und in der Beurteilung der Lagebeziehung des Tumors zu den komplexen anatomischen Strukturen wie Hirnnerven und

  4. Electrophysiological Monitoring in Patients With Tumors of the Skull Base Treated by Carbon-12 Radiation Therapy

    Energy Technology Data Exchange (ETDEWEB)

    Carozzo, Simone [Department of Neuroscience, Ophthalmology, and Genetics, University of Genova, Genova (Italy); Schardt, Dieter [Department of Biophysics, GSI Helmholtzzentrum für Schwerionenforschung, Darmstadt (Germany); Narici, Livio [Department of Physics, University of Rome Tor Vergata, Rome (Italy); Combs, Stephanie E.; Debus, Jürgen [Department of Radiation Oncology, University of Heidelberg, Heidelberg (Germany); Sannita, Walter G., E-mail: [Department of Neuroscience, Ophthalmology, and Genetics, University of Genova, Genova (Italy); Department of Psychiatry, State University of New York, Stony Brook, New York (United States)


    Purpose: To report the results of short-term electrophysiologic monitoring of patients undergoing {sup 12}C therapy for the treatment of skull chordomas and chondrosarcomas unsuitable for radical surgery. Methods and Materials: Conventional electroencephalogram (EEG) and retinal and cortical electrophysiologic responses to contrast stimuli were recorded from 30 patients undergoing carbon ion radiation therapy, within a few hours before the first treatment and after completion of therapy. Methodologies and procedures were compliant with the guidelines of the International Federation for Clinical Neurophysiology and International Society for Clinical Electrophysiology of Vision. Results: At baseline, clinical signs were reported in 56.6% of subjects. Electrophysiologic test results were abnormal in 76.7% (EEG), 78.6% (cortical evoked potentials), and 92.8% (electroretinogram) of cases, without correlation with neurologic signs, tumor location, or therapy plan. Results on EEG, but not electroretinograms and cortical responses, were more often abnormal in patients with reported clinical signs. Abnormal EEG results and retinal/cortical responses improved after therapy in 40% (EEG), 62.5% (cortical potentials), and 70% (electroretinogram) of cases. Results on EEG worsened after therapy in one-third of patients whose recordings were normal at baseline. Conclusions: The percentages of subjects whose EEG results improved or worsened after therapy and the improvement of retinal/cortical responses in the majority of patients are indicative of a limited or negligible (and possibly transient) acute central nervous system toxicity of carbon ion therapy, with a significant beneficial effect on the visual pathways. Research on large samples would validate electrophysiologic procedures as a possible independent test for central nervous system toxicity and allow investigation of the correlation with clinical signs; repeated testing over time after therapy would demonstrate, and may

  5. Encouraging Early Clinical Outcomes With Helical Tomotherapy-Based Image-Guided Intensity-Modulated Radiation Therapy for Residual, Recurrent, and/or Progressive Benign/Low-Grade Intracranial Tumors: A Comprehensive Evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Gupta, Tejpal, E-mail: [Department of Radiation Oncology, ACTREC/TMH, Tata Memorial Centre, Kharghar, Navi Mumbai (India); Wadasadawala, Tabassum; Master, Zubin; Phurailatpam, Reena; Pai-Shetty, Rajershi; Jalali, Rakesh [Department of Radiation Oncology, ACTREC/TMH, Tata Memorial Centre, Kharghar, Navi Mumbai (India)


    Purpose: To report early clinical outcomes of helical tomotherapy (HT)-based image-guided intensity-modulated radiation therapy (IMRT) in brain tumors of varying shape, size, and location. Materials and Methods: Patients with residual, recurrent, and/or progressive low-grade intracranial and skull-base tumors were treated on a prospective protocol of HT-based IMRT and followed clinicoradiologically. Standardized metrics were used for plan evaluation and outcome analysis. Results: Twenty-seven patients with 30 lesions were treated to a median radiotherapy dose of 54 Gy in 30 fractions. All HT plans resulted in excellent target volume coverage with steep dose-gradients. The mean (standard deviation) dose homogeneity index and conformity index was 0.07 (0.05) and 0.71 (0.08) respectively. At first response assessment, 20 of 30 lesions were stable, whereas 9 showed partial regression. One patient with a recurrent clival chordoma though neurologically stable showed imaging-defined progression, whereas another patient with stable disease on serial imaging had sustained neurologic worsening. With a median follow-up of 19 months (interquartile range, 11-26 months), the 2-year clinicoradiological progression-free survival and overall survival was 93.3% and 100% respectively. Conclusions: Careful selection of radiotherapy technique is warranted for benign/low-grade brain tumors to achieve durable local control with minimum long-term morbidity. Large or complex-shaped tumors benefit most from IMRT. Our early clinical experience of HT-based IMRT for brain tumors has been encouraging.

  6. Dosimetric comparisons of carbon ion treatment plans for 1D and 2D ripple filters with variable thicknesses (United States)

    Printz Ringbæk, Toke; Weber, Uli; Santiago, Alina; Simeonov, Yuri; Fritz, Peter; Krämer, Michael; Wittig, Andrea; Bassler, Niels; Engenhart-Cabillic, Rita; Zink, Klemens


    A ripple filter (RiFi)—also called mini-ridge filter—is a passive energy modulator used in particle beam treatments that broadens the Bragg peak (BP) as a function of its maximum thickness. The number of different energies requested from the accelerator can thus be reduced, which significantly reduces the treatment time. A new second generation RiFi with 2D groove shapes was developed using rapid prototyping, which optimizes the beam-modulating material and enables RiFi thicknesses of up to 6 mm. Carbon ion treatment plans were calculated using the standard 1D 3 mm thick RiFi and the new 4 and 6 mm 2D RiFis for spherical planning target volumes (PTVs) in water, eight stage I non-small cell lung cancer cases, four skull base chordoma cases and three prostate cancer cases. TRiP98 was used for treatment planning with facility-specific base data calculated with the Monte Carlo code SHIELD-HIT12A. Dose-volume-histograms, spatial dose distributions and dosimetric indexes were used for plan evaluation. Plan homogeneity and conformity of thinner RiFis were slightly superior to thicker RiFis but satisfactory results were obtained for all RiFis investigated. For the 6 mm RiFi, fine structures in the dose distribution caused by the larger energy steps were observed at the PTV edges, in particular for superficial and/or very small PTVs but performances for all RiFis increased with penetration depth due to straggling and scattering effects. Plans with the new RiFi design yielded for the studied cases comparable dosimetric results to the standard RiFi while the 4 and 6 mm RiFis lowered the irradiation time by 25-30% and 45-49%, respectively.

  7. Typical tumors of the petrous bone; Typische Tumoren des Felsenbeins

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    Ahlhelm, F.; Mueller, U. [Kantonsspital Baden AG, Abteilung fuer Neuroradiologie, Institut fuer Radiologie, Baden (Switzerland); Ulmer, S. [Medizinisch-Radiologisches Institut, Zuerich (Switzerland)


    In the region of the petrous bone, inner acoustic canal and cerebellopontine angle, a variety of different tissues can be found, such as bony, epithelial, neural and vascular structures. Tumorous or tumor-like lesions, vascular or bony malformations or other pathologies can therefore be found in all of these areas. We discuss various frequently occurring tumorous or tumor-like pathologies including congential lesions, such as mucoceles, inflammatory disorders including osteomyelitis, pseudotumors and Wegener's granulomatosis. Benign non-neoplastic lesions, such as cholesteatoma, cholesterol granuloma, epidermoid and benign neoplastic tumors, such as the most commonly found vestibular schwannoma, meningeoma, paraganglioma, vascular pathologies and finally malignant lesions, such as metastasis, chordoma or chondrosarcoma and endolymphatic sac tumor (ELST) are also discussed. The emphasis of this article is on the appearance of these entities in computed tomography (CT) and more so magnetic resonance imaging (MRI), it provides key facts and typical images and discusses possibilities how to distinguish these pathologies. (orig.) [German] In der Region des Felsenbein, inneren Gehoerkanals und Kleinhirnbrueckenwinkels findet sich eine Vielzahl an unterschiedlichen Gewebearten inklusive knoechernes, epitheliales, nervales und vaskulaeres Gewebe. Tumoren oder tumoraehnliche Laesionen, ossaere oder vaskulaere Pathologien koennen entsprechend dort gefunden werden. Wir diskutieren verschiedene Tumoren oder tumoraehnliche Pathologien inklusive angeborene Laesionen wie Muko- und Meningozelen, entzuendliche Veraenderungen wie die Osteomyelitis, Pseudotumoren, die Wegener-Granulomatose, nichtneoplastische Tumoren wie das Epidermoid, Cholesteatom oder Cholesterolgranulom und gutartige neoplastische Tumoren wie das am haeufigsten zu findende Vestibularisschwannom, das Paragangliom und das Meningeom, Gefaessprozesse/-pathologien und schliesslich maligne Laesionen wie Metastasen

  8. Primary sarcomas of the central nervous system: UCSF experience (1985-2005

    Directory of Open Access Journals (Sweden)

    Tarık TİHAN


    Full Text Available Sarcomas constitute less than 2% of all cancers, and are a highly diverse group of neoplasms. Primary sarcomas of the central nervous system (CNS are even less common, and our experience is limited by lack of studies with sufficient size that can address the challenges in predicting behavior or management. It is critical to recognize the characteristics of these uncommon neoplasms and to develop better predictors for prognosis and behavior.We have conducted a search of the UCSF Department of Pathology and UCSF Cancer Center Registry databases for all primary CNS sarcomas that were diagnosed and treated between 1985 and 2005. Hemangiopericytomas were included, so were the solitary fibrous tumors due to their close association with the former. We excluded all cases of metastatic sarcomas, chordomas, sarcomatoid variants of all neuroepithelial neoplasms, Ewing’s sarcomas and other embryonal tumors. In addition, we have identified all soft tissue sarcomas diagnosed and treated during the same period. There were 43 primary CNS neoplasms that fulfilled the inclusion criteria. At the same time, we identified 1706 sarcomas primary to the soft tissue. Primary CNS sarcomas included 16 hemangiopericytomas, 15 chondrosarcomas, 3 solitary fibrous tumors, 3 osteosarcomas, 2 leiomyosarcomas, 2 undifferentiated sarcomas, 1 histiocytic sarcoma, and 1 fibrosarcoma. There was a distinctly higher frequency of hemangiopericytoma in the CNS compared to soft tissue. In addition, a group of low grade, parasagittal chondrosarcomas were noted for their highly indolent biological behavior. Unlike some previous series, our cohort was devoid of angiosarcoma and malignant fibrous histiocytoma. This study underscores the limitations of single institutional series, and highlights the value of multi-institutional studies to understand and better treat primary CNS sarcomas.

  9. A panoramic view of the skull base: systematic review of open and endoscopic endonasal approaches to four tumors. (United States)

    Graffeo, Christopher S; Dietrich, August R; Grobelny, Bartosz; Zhang, Meng; Goldberg, Judith D; Golfinos, John G; Lebowitz, Richard; Kleinberg, David; Placantonakis, Dimitris G


    Endoscopic endonasal surgery has been established as the safest approach to pituitary tumors, yet its role in other common skull base lesions has not been established. To answer this question, we carried out a systematic review of reported series of open and endoscopic endonasal approaches to four major skull base tumors: olfactory groove meningiomas (OGM), tuberculum sellae meningiomas (TSM), craniopharyngiomas (CRA), and clival chordomas (CHO). Data from 162 studies containing 5,701 patients were combined and compared for differences in perioperative mortality, gross total resection (GTR), cerebrospinal fluid (CSF) leak, neurological morbidity, post-operative visual function, post-operative anosmia, post-operative diabetes insipidus (DI), and post-operative obesity/hyperphagia. Weighted average rates for each outcome were calculated using relative study size. Our findings indicate similar rates of GTR and perioperative mortality between open and endoscopic approaches for all tumor types. CSF leak was increased after endoscopic surgery. Visual function symptoms were more likely to improve after endoscopic surgery for TSM, CRA, and CHO. Post-operative DI and obesity/hyperphagia were significantly increased after open resection in CRA. Recurrence rates per 1,000 patient-years of follow-up were higher in endoscopy for OGM, TSM, and CHO. Trends for open and endoscopic surgery suggested modest improvement in all outcomes over time. Our observations suggest that endonasal endoscopy is a safe alternative to craniotomy and may be preferred for certain tumor types. However, endoscopic surgery is associated with higher rates of CSF leak, and possibly increased recurrence rates. Prospective study with long-term follow-up is required to verify these preliminary observations.

  10. Dosimetric comparisons of carbon ion treatment plans for 1D and 2D ripple filters with variable thicknesses. (United States)

    Ringbæk, Toke Printz; Weber, Uli; Santiago, Alina; Simeonov, Yuri; Fritz, Peter; Krämer, Michael; Wittig, Andrea; Bassler, Niels; Engenhart-Cabillic, Rita; Zink, Klemens


    A ripple filter (RiFi)-also called mini-ridge filter-is a passive energy modulator used in particle beam treatments that broadens the Bragg peak (BP) as a function of its maximum thickness. The number of different energies requested from the accelerator can thus be reduced, which significantly reduces the treatment time. A new second generation RiFi with 2D groove shapes was developed using rapid prototyping, which optimizes the beam-modulating material and enables RiFi thicknesses of up to 6 mm. Carbon ion treatment plans were calculated using the standard 1D 3 mm thick RiFi and the new 4 and 6 mm 2D RiFis for spherical planning target volumes (PTVs) in water, eight stage I non-small cell lung cancer cases, four skull base chordoma cases and three prostate cancer cases. TRiP98 was used for treatment planning with facility-specific base data calculated with the Monte Carlo code SHIELD-HIT12A. Dose-volume-histograms, spatial dose distributions and dosimetric indexes were used for plan evaluation. Plan homogeneity and conformity of thinner RiFis were slightly superior to thicker RiFis but satisfactory results were obtained for all RiFis investigated. For the 6 mm RiFi, fine structures in the dose distribution caused by the larger energy steps were observed at the PTV edges, in particular for superficial and/or very small PTVs but performances for all RiFis increased with penetration depth due to straggling and scattering effects. Plans with the new RiFi design yielded for the studied cases comparable dosimetric results to the standard RiFi while the 4 and 6 mm RiFis lowered the irradiation time by 25-30% and 45-49%, respectively.

  11. Indications of Carbon Ion Therapy at CNAO (United States)

    Orecchia, Roberto; Rossi, Sandro; Fossati, Piero


    CNAO will be a dual center capable of providing therapeutic beams of protons and carbon ions with maximum energy of 400 MeV/u. At the beginning, it will be equipped with three treatment rooms with fixed horizontal and vertical beam lines. In a subsequent phase, two more rooms with a rotating gantry are foreseen. An active spot scanning dose delivery system will be employed. Initially, 80% of the treatments will be carried out with carbon ions. All patients will be treated within clinical trials to assess carbon ion indications with an evidence-based methodology. Seven disease-specific working groups have been developed: lung tumors, liver tumors, sarcomas, head and neck tumors, central nervous system lesions, eye tumors and pediatric tumors. The last two groups will be treated mainly with protons. In the first phase, CNAO will focus on head and neck cancers, treating inoperable, residual or recurrent malignant salivary gland tumors, mucosal melanoma, adenocarcinoma and unfavorably located SCC (nasal and paranasal sinuses). Carbon ions will be employed as a boost in the treatment of locally advanced, poor prognosis, SCC of the hypopharynx and tongue base. Bone and soft tissue sarcomas of the extremity will be treated with a limb-sparing approach, and trunk sarcomas will be treated with exclusive or post-operative irradiation. Skull base tumors (chordoma and chondrosarcoma), recurrent or malignant meningioma and glial tumors will be treated with carbon ions. After sufficient expertise has been gained in coping with organ motion, CNAO will start treating thoracic and abdominal targets. HCC will be treated in inoperable patients with one or more lesions that can be included in a single CTV. Early stage NSCLC will be treated. In the second phase, two more groups on gynecological malignancies and digestive tumors (esophageal cancer, rectal cancer, pancreatic cancer) will be created.

  12. Risk factors for postoperative cerebrospinal fluid leak and meningitis after expanded endoscopic endonasal surgery. (United States)

    Ivan, Michael E; Iorgulescu, J Bryan; El-Sayed, Ivan; McDermott, Michael W; Parsa, Andrew T; Pletcher, Steven D; Jahangiri, Arman; Wagner, Jeffrey; Aghi, Manish K


    Postoperative cerebrospinal fluid (CSF) leak is a serious complication of transsphenoidal surgery, which can lead to meningitis and often requires reparative surgery. We sought to identify preoperative risk factors for CSF leaks and meningitis. We reviewed 98 consecutive expanded endoscopic endonasal surgeries performed from 2008-2012 and analyzed preoperative comorbidities, intraoperative techniques, and postoperative care. Univariate and multivariate analyses were performed. The most common pathologies addressed included pituitary adenoma, Rathke cyst, chordoma, esthesioneuroblastoma, meningioma, nasopharyngeal carcinoma, and squamous cell carcinoma. There were 11 CSF leaks (11%) and 10 central nervous system (CNS) infections (10%). Univariate and multivariate analysis of preoperative risk factors showed that patients with non-ideal body mass index (BMI) were associated with higher rate of postoperative CSF leak and meningitis (both p<0.01). Also, patients with increasing age were associated with increased CSF leak (p = 0.03) and the length of time a lumbar drain was used postoperatively was associated with infection in a univariate analysis. In addition, three of three endoscopic transsphenoidal surgeries combined with open cranial surgery had a postoperative CSF leak and CNS infection rate which was a considerably higher rate than for transsphenoidal surgeries alone or surgeries staged with open cases (p<0.01 and p=0.04, respectively) In this series of expanded endoscopic transsphenoidal surgeries, preoperative BMI remains the most important preoperative predictor for CSF leak and infection. Other risk factors include age, intraoperative CSF leak, lumbar drain duration, and cranial combined cases. Risks associated with complex surgical resections when combining open and endoscopic approaches could be minimized by staging these procedures.

  13. 内分泌腺来源的血管内皮生长因子在脊柱肿瘤组织中的表达%Study of the expression of endocrine gland-derived vascular endothelial growth factor in the spinal cord tumors

    Institute of Scientific and Technical Information of China (English)

    陈学武; 杨评山; 潘光杰


    Objective To explore the relationship of spinal tumors and endocrine gland-derived vascular endothelial growth factor( EG-VEGF) .Methods 77 cases of spinal tumors and 17 cases of vertebral compression fractures were collected.The testing group were grouped according to the pathological type of spinal tumor,the observ-ing group was consisted of vertebral compression fracture cases.The samples of various types of tumors in patients were collected during operation and the positive rate and expression of EG-VEGF mRNA were detected by RT-PCR technique,and the results of each group were statistical analysed.Results The results of relative expression of EG-VEGF mRNA in the unknown primary spinal metastases group,osteosarcoma group and chordoma group were higher, there were no statistically significant difference compared between three groups(all P>0.05).Relative expressions of that in osteoid osteoma group and giant cell tumor group were lower,so was in the observation group,there were no sig-nificant differences in three groups ( all P>0.05);The relative expressions of EG-VEGF mRNA in CUP group, osteosarcoma group and chordoma group were significantly higher than the osteoid osteoma group,with statistical sig-nificance[(0.81 ±0.21),(0.84 ±0.22),(0.79 ±0.41)vs(0.53 ±0.19),t=0.86,0.82,0.81,all P<0.05]. Similar results were also found in EG -VEGF mRNA positive expression rate in three groups.Conclusion EG-VEGF in normal spine spinal vertebrae and primary benign tumor ( osteoid osteoma,giant cell tumor) showed lower expression,which consistent with its tissue specificity,but in primary malignant spinal cancer ( osteosarcoma, chordoma) and unknown primary tumor spinal metastases showed the higher expression, indicating that it may be a measure of an important indicator of spinal cancer, and may play a key role in early diagnosis and treatment of cancer of the spine in the future.%目的:探索脊柱肿瘤与内分泌腺来源的血管内皮生长因子( EG-VEGF)的

  14. Single-stage en bloc resection using a posterior approach for sacral tumors

    Institute of Scientific and Technical Information of China (English)

    Zhong Fang; Wei Wu; Wei Xiong; Guanghui Li; Hui Liao; Jun Xiao; Fengjing Guo; Anmin Chen; Feng Li 


    Objective This study aims to investigate the surgical results of single-stage en bloc resections using a posterior approach for sacral tumors and evaluate its benefit for these tumors. Methods A total of 32 cases involving patients with sacral tumors who were treated with single-stage en bloc resection by posterior approach from April 2004 to January 2010 were reviewed. The patient cohort included 20 males and 12 females. The average patient age was 49.1 years old (range, 18 to 75 years old). Twenty-seven patients suf ered from primary sacral tumors, including 17 patients with chordomas, 5 patients with giant cel tumors of the bone, 3 patients with chondrosarcoma, 1 patient with sarcoma of the nerve fibers, and 1 patient with neurofibromatosis. Five patients were diagnosed with sacral metastatic tumors, including 2 cases of breast cancer, 1 case of renal carcinoma, 1 case of thyroid cancer, and 1 case of lung cancer. According to the Frankel grading system, there were 3 Grade B tumors, 4 Grade C tumors, 10 Grade D tumors, and 15 Grade E tumors. Results The operation took 265 min on average (range, 130–360 min), and blood loss was 1676 mL on average (range, 800–1800 mL) during the operation. The fol ow-up period ranged from 6 months to 6.2 years. Al patients had pain prior to operation. Twenty-eight patients experienced complete pain-relief, and 4 patients experienced partial pain-relief after their operations. In al patients, neurological function was improved more than one grade using the Frankel grading system. Up to now, 5 patients experienced local recurrence after operation, and 2 patients were deceased. The remaining patients are stil alive without recurrence. Conclusion Single-stage en bloc resection through a posterior approach for sacral tumors is feasible, safe, and ef ective. It has many advantages, such as control ing local recurrence, thorough decompression of the spinal cord, relieving pain, improving quality of life, and prolonging survival.

  15. Dose–Volume Relationships Associated With Temporal Lobe Radiation Necrosis After Skull Base Proton Beam Therapy

    Energy Technology Data Exchange (ETDEWEB)

    McDonald, Mark W., E-mail: [Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana (United States); Indiana University Health Proton Therapy Center, Bloomington, Indiana (United States); Linton, Okechukwu R. [Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana (United States); Calley, Cynthia S.J. [Department of Biostatistics, Indiana University School of Medicine, Indianapolis, Indiana (United States)


    Purpose: We evaluated patient and treatment parameters correlated with development of temporal lobe radiation necrosis. Methods and Materials: This was a retrospective analysis of a cohort of 66 patients treated for skull base chordoma, chondrosarcoma, adenoid cystic carcinoma, or sinonasal malignancies between 2005 and 2012, who had at least 6 months of clinical and radiographic follow-up. The median radiation dose was 75.6 Gy (relative biological effectiveness [RBE]). Analyzed factors included gender, age, hypertension, diabetes, smoking status, use of chemotherapy, and the absolute dose:volume data for both the right and left temporal lobes, considered separately. A generalized estimating equation (GEE) regression analysis evaluated potential predictors of radiation necrosis, and the median effective concentration (EC50) model estimated dose–volume parameters associated with radiation necrosis. Results: Median follow-up time was 31 months (range 6-96 months) and was 34 months in patients who were alive. The Kaplan-Meier estimate of overall survival at 3 years was 84.9%. The 3-year estimate of any grade temporal lobe radiation necrosis was 12.4%, and for grade 2 or higher radiation necrosis was 5.7%. On multivariate GEE, only dose–volume relationships were associated with the risk of radiation necrosis. In the EC50 model, all dose levels from 10 to 70 Gy (RBE) were highly correlated with radiation necrosis, with a 15% 3-year risk of any-grade temporal lobe radiation necrosis when the absolute volume of a temporal lobe receiving 60 Gy (RBE) (aV60) exceeded 5.5 cm{sup 3}, or aV70 > 1.7 cm{sup 3}. Conclusions: Dose–volume parameters are highly correlated with the risk of developing temporal lobe radiation necrosis. In this study the risk of radiation necrosis increased sharply when the temporal lobe aV60 exceeded 5.5 cm{sup 3} or aV70 > 1.7 cm{sup 3}. Treatment planning goals should include constraints on the volume of temporal lobes receiving

  16. 斜坡异位泌乳素垂体腺瘤 1 例临床分析并文献复习%Clinical analysis of one patient with ectopic prolactinoma in clivus and review of literatures

    Institute of Scientific and Technical Information of China (English)

    穆林森; 张红波; 陈谦学; 桂松柏; 曾小君; 白吉伟; 李储忠; 赵鹏; 孙彦辉


    Objective To explore the diagnosis , differential diagnosis and treatment of ectopic prolactinoma in clivus .Methods The clinical data of one patient with ectopic pituitary adenoma ( EPA) in clivus underwent surgery and pathology results was retrospectively analyzed ,and the clinical features with literature review were summarized .Results The cases was diagnosed as hyperprolactinemia ,MRI showed clivus mass and preoperative diagnosis was chordoma .The tumors were totally resected through transendoscopic single nostril transsphenoidal approach .Pathological immunohistochemical test showed prolactin positive , the hyperprolactinemia not returned to normal after surgery , oral bromocriptine treatment to control it , follow-up of 1 year without recurrence . Conclusions Ectopic clivus pituitary adenoma was rarely in clinic and easily be misdiagnosed .We should continue to adopt a comprehensive treatment based on surgery .%目的 探讨斜坡异位泌乳素垂体腺瘤的诊断与鉴别诊断及治疗方法. 方法 回顾性分析1例经手术及术后病理检查确诊的斜坡异位垂体腺瘤患者的临床资料,并结合文献总结其临床特点. 结果 该例患者表现为高泌乳素血症,MRI检查发现斜坡病变,术前诊断为脊索瘤. 采用内镜下单鼻孔经鼻蝶入路显微手术全切除肿瘤. 肿瘤病理免疫组化检查泌乳素阳性,术后未完全降至正常,口服溴隐亭治疗,随访1年无复发. 结论 斜坡异位垂体腺瘤临床罕见,误诊率高;仍应采用以手术为主的综合治疗.

  17. Small bowel toxicity after high dose spot scanning-based proton beam therapy for paraspinal/retroperitoneal neoplasms

    Energy Technology Data Exchange (ETDEWEB)

    Schneider, R.A.; Albertini, F.; Koch, T.; Ares, C.; Lomax, A.; Goitein, G. [Paul Scherrer Institute PSI, Villigen (Switzerland). Center for Proton Therapy; Vitolo, V. [Fondazione CNAO, Pavia (Italy); Hug, E.B. [Paul Scherrer Institute PSI, Villigen (Switzerland). Center for Proton Therapy; ProCure Proton Therapy Centers, New York, NY (United States)


    Purpose: Mesenchymal tumours require high-dose radiation therapy (RT). Small bowel (SB) dose constraints have historically limited dose delivery to paraspinal and retroperitoneal targets. This retrospective study correlated SB dose-volume histograms with side-effects after proton radiation therapy (PT). Patients and methods: Between 1997 and 2008, 31 patients (mean age 52.1 years) underwent spot scanning-based PT for paraspinal/retroperitoneal chordomas (81 %), sarcomas (16 %) and meningiom (3 %). Mean total prescribed dose was 72.3 Gy (relative biologic effectiveness, RBE) delivered in 1.8-2 Gy (RBE) fractions. Mean follow-up was 3.8 years. Based on the pretreatment planning CT, SB dose distributions were reanalysed. Results: Planning target volume (PTV) was defined as gross tumour volume (GTV) plus 5-7 mm margins. Mean PTV was 560.22 cm{sup 3}. A mean of 93.2 % of the PTV was covered by at least 90 % of the prescribed dose. SB volumes (cm{sup 3}) receiving doses of 5, 20, 30, 40, 50, 60, 70, 75 and 80 Gy (RBE) were calculated to give V5, V20, V30, V40, V50, V60, V70, V75 and V80 respectively. In 7/31 patients, PT was accomplished without any significant SB irradiation (V5 = 0). In 24/31 patients, mean maximum dose (Dmax) to SB was 64.1 Gy (RBE). Despite target doses of > 70 Gy (RBE), SB received > 50 and > 60 Gy (RBE) in only 61 and 54 % of patients, respectively. Mean SB volumes (cm{sup 3}) covered by different dose levels (Gy, RBE) were: V20 (n = 24): 45.1, V50 (n = 19): 17.7, V60 (n = 17): 7.6 and V70 (n = 12): 2.4. No acute toxicity {>=} grade 2 or late SB sequelae were observed. Conclusion: Small noncircumferential volumes of SB tolerated doses in excess of 60 Gy (RBE) without any clinically-significant late adverse effects. This small retrospective study has limited statistical power but encourages further efforts with higher patient numbers to define and establish high-dose threshold models for SB toxicity in modern radiation oncology. (orig.)

  18. Impact of tissue specific parameters on the predition of the biological effectiveness for treatment planning in ion beam therapy

    Energy Technology Data Exchange (ETDEWEB)

    Gruen, Rebecca Antonia


    Treatment planning in ion beam therapy requires a reliable estimation of the relative biological effectiveness (RBE) of the irradiated tissue. For the pilot project at GSI Helmholtzzentrum fuer Schwerionenforschung GmbH and at other European ion beam therapy centers RBE prediction is based on a biophysical model, the Local Effect Model (LEM). The model version in use, LEM I, is optimized to give a reliable estimation of RBE in the target volume for carbon ion irradiation. However, systematic deviations are observed for the entrance channel of carbon ions and in general for lighter ions. Thus, the LEM has been continuously developed to improve accuracy. The recent version LEM IV has proven to better describe in-vitro cell experiments. Thus, for the clinical application of LEM IV it is of interest to analyze potential differences compared to LEM I under treatment-like conditions. The systematic analysis presented in this work is aiming at the comparison of RBE-weighted doses resulting from different approaches and model versions for protons and carbon ions. This will facilitate the assessment of consequences for clinical application and the interpretation of clinical results from different institutions. In the course of this thesis it has been shown that the RBE-weighted doses predicted on the basis of LEM IV for typical situations representing chordoma treatments differ on average by less than 10 % to those based on LEM I and thus also allow a consistent interpretation of the clinical results. At Japanese ion beam therapy centers the RBE is estimated using their clinical experience from neutron therapy in combination with in-vitro measurements for carbon ions (HIMAC approach). The methods presented in this work allow direct comparison of the HIMAC approach and the LEM and thus of the clinical results obtained at Japanese and European ion beam therapy centers. Furthermore, the sensitivity of the RBE on the model parameters was evaluated. Among all parameters the

  19. Temporal Lobe Reactions After Carbon Ion Radiation Therapy: Comparison of Relative Biological Effectiveness–Weighted Tolerance Doses Predicted by Local Effect Models I and IV

    Energy Technology Data Exchange (ETDEWEB)

    Gillmann, Clarissa, E-mail: [Department of Radiation Oncology and Radiation Therapy, Heidelberg University Hospital, Heidelberg (Germany); Jäkel, Oliver [Department of Radiation Oncology and Radiation Therapy, Heidelberg University Hospital, Heidelberg (Germany); Heidelberg Ion Beam Therapy Center (HIT), Heidelberg (Germany); Department of Medical Physics in Radiation Oncology, German Cancer Research Center (DKFZ), Heidelberg (Germany); Schlampp, Ingmar [Department of Radiation Oncology and Radiation Therapy, Heidelberg University Hospital, Heidelberg (Germany); Karger, Christian P. [Department of Medical Physics in Radiation Oncology, German Cancer Research Center (DKFZ), Heidelberg (Germany)


    Purpose: To compare the relative biological effectiveness (RBE)–weighted tolerance doses for temporal lobe reactions after carbon ion radiation therapy using 2 different versions of the local effect model (LEM I vs LEM IV) for the same patient collective under identical conditions. Methods and Materials: In a previous study, 59 patients were investigated, of whom 10 experienced temporal lobe reactions (TLR) after carbon ion radiation therapy for low-grade skull-base chordoma and chondrosarcoma at Helmholtzzentrum für Schwerionenforschung (GSI) in Darmstadt, Germany in 2002 and 2003. TLR were detected as visible contrast enhancements on T1-weighted MRI images within a median follow-up time of 2.5 years. Although the derived RBE-weighted temporal lobe doses were based on the clinically applied LEM I, we have now recalculated the RBE-weighted dose distributions using LEM IV and derived dose-response curves with Dmax,V-1 cm³ (the RBE-weighted maximum dose in the remaining temporal lobe volume, excluding the volume of 1 cm³ with the highest dose) as an independent dosimetric variable. The resulting RBE-weighted tolerance doses were compared with those of the previous study to assess the clinical impact of LEM IV relative to LEM I. Results: The dose-response curve of LEM IV is shifted toward higher values compared to that of LEM I. The RBE-weighted tolerance dose for a 5% complication probability (TD{sub 5}) increases from 68.8 ± 3.3 to 78.3 ± 4.3 Gy (RBE) for LEM IV as compared to LEM I. Conclusions: LEM IV predicts a clinically significant increase of the RBE-weighted tolerance doses for the temporal lobe as compared to the currently applied LEM I. The limited available photon data do not allow a final conclusion as to whether RBE predictions of LEM I or LEM IV better fit better clinical experience in photon therapy. The decision about a future clinical application of LEM IV therefore requires additional analysis of temporal lobe reactions in a

  20. D-IMRT verification with a 2D pixel ionization chamber: dosimetric and clinical results in head and neck cancer (United States)

    Stasi, M.; Giordanengo, S.; Cirio, R.; Boriano, A.; Bourhaleb, F.; Cornelius, I.; Donetti, M.; Garelli, E.; Gomola, I.; Marchetto, F.; Porzio, M.; Sanz Freire, C. J.; Sardo, A.; Peroni, C.


    Dynamic intensity-modulated radiotherapy (D-IMRT) using the sliding-window technique is currently applied for selected treatments of head and neck cancer at Institute for Cancer Research and Treatment of Candiolo (Turin, Italy). In the present work, a PiXel-segmented ionization Chamber (PXC) has been used for the verification of 19 fields used for four different head and neck cancers. The device consists of a 32 × 32 matrix of 1024 parallel-plate ionization chambers arranged in a square of 24 × 24 cm2 area. Each chamber has 0.4 cm diameter and 0.55 cm height; a distance of 0.75 cm separates the centre of adjacent chambers. The sensitive volume of each single ionization chamber is 0.07 cm3. Each of the 1024 independent ionization chambers is read out with a custom microelectronics chip. The output factors in water obtained with the PXC at a depth of 10 cm were compared to other detectors and the maximum difference was 1.9% for field sizes down to 3 × 3 cm2. Beam profiles for different field dimensions were measured with the PXC and two other types of ionization chambers; the maximum distance to agreement (DTA) in the 20-80% penumbra region of a 3 × 3 cm2 field was 0.09 cm. The leaf speed of the multileaf collimator was varied between 0.07 and 2 cm s-1 and the detector response was constant to better than 0.6%. The behaviour of the PXC was measured while varying the dose rate between 0.21 and 1.21 Gy min-1; the mean difference was 0.50% and the maximum difference was 0.96%. Using fields obtained with an enhanced dynamic wedge and a staircase-like (step) IMRT field, the PXC has been tested for simple 1D modulated beams; comparison with film gave a maximum DTA of 0.12 cm. The PXC was then used to check four different IMRT plans for head and neck cancer treatment: cervical chordoma, parotid, ethmoid and skull base. In the comparison of the PXC versus film and PXC versus treatment planning system, the number of pixels with γ parameter <=1 was 97.7% and 97

  1. D-IMRT verification with a 2D pixel ionization chamber: dosimetric and clinical results in head and neck cancer

    Energy Technology Data Exchange (ETDEWEB)

    Stasi, M [Institute for Cancer Research and Treatment (IRCC), Candiolo and A.S.O. Ordine Maurizano, Torino (Italy); Giordanengo, S [Istituto Nazionale di Fisica Nucleare (INFN), Via P. Giuria 1, I-10125 Torino (Italy); Cirio, R [Istituto Nazionale di Fisica Nucleare (INFN), Via P. Giuria 1, I-10125 Torino (Italy)] [and others


    Dynamic intensity-modulated radiotherapy (D-IMRT) using the sliding-window technique is currently applied for selected treatments of head and neck cancer at Institute for Cancer Research and Treatment of Candiolo (Turin, Italy). In the present work, a PiXel-segmented ionization Chamber (PXC) has been used for the verification of 19 fields used for four different head and neck cancers. The device consists of a 32 x 32 matrix of 1024 parallel-plate ionization chambers arranged in a square of 24 x 24 cm{sup 2} area. Each chamber has 0.4 cm diameter and 0.55 cm height; a distance of 0.75 cm separates the centre of adjacent chambers. The sensitive volume of each single ionization chamber is 0.07 cm{sup 3}. Each of the 1024 independent ionization chambers is read out with a custom microelectronics chip. The output factors in water obtained with the PXC at a depth of 10 cm were compared to other detectors and the maximum difference was 1.9% for field sizes down to 3 x 3 cm{sup 2}. Beam profiles for different field dimensions were measured with the PXC and two other types of ionization chambers; the maximum distance to agreement (DTA) in the 20-80% penumbra region of a 3 x 3 cm{sup 2} field was 0.09 cm. The leaf speed of the multileaf collimator was varied between 0.07 and 2 cm s{sup -1} and the detector response was constant to better than 0.6%. The behaviour of the PXC was measured while varying the dose rate between 0.21 and 1.21 Gy min{sup -1}; the mean difference was 0.50% and the maximum difference was 0.96%. Using fields obtained with an enhanced dynamic wedge and a staircase-like (step) IMRT field, the PXC has been tested for simple 1D modulated beams; comparison with film gave a maximum DTA of 0.12 cm. The PXC was then used to check four different IMRT plans for head and neck cancer treatment: cervical chordoma, parotid, ethmoid and skull base. In the comparison of the PXC versus film and PXC versus treatment planning system, the number of pixels with {gamma

  2. Seguimiento radiológico en paciente con fibroma condromixoide del seno frontal: Diagnóstico diferencial, alternativas terapéuticas y revisión de la literatura Radiological follow-up of a patient with chondromyxoid fibroma of the frontal sinus: Differential diagnosis, therapeutic alternatives and a revision of the literature

    Directory of Open Access Journals (Sweden)

    I. Zubillaga Rodriguez


    Full Text Available Introducción. El fibroma condromixoide es un tumor benigno de crecimiento lento y poco frecuente. Su incidencia en la región craneofacial es rara, especialmente en el seno frontal. Objetivos. Presentamos el seguimiento a largo plazo de un paciente intervenido en relación a un fibroma condromixoide del seno frontal. Se discuten los distintos diagnósticos diferenciales y las alternativas terapéuticas. Discusión. Desde el punto de vista de anatomía patológica es necesario hacer el diagnóstico diferencial con condrosarcoma, mixosarcoma, mixoma y cordoma. Desde el punto de vista radiológico, el diagnóstico de fibroma condromixoide ha de ser considerado en toda lesión ósea aislada con expansión u osteolisis, márgenes lobulados y septos intralesionales. El tratamiento del fibroma condromixoide incluye principalmente curetaje o resección en bloque, mientras que la radioterapia aislada no se considera de primera elección. Conclusiones. La naturaleza benigna del fibroma condromixoide implica la importancia de evitar tratamientos radicales. El abordaje subcraneal se presenta como una alternativa válida para el tratamiento de lesiones localizadas en el seno frontal. Es fundamental un seguimiento clínico-radiológico adecuado para identificar precozmente posibles recidivas.Introduction. Chondromyxoid fibroma is a slow growing and rare benign bone tumor. Its location in the craniofacial skeleton is not common, especially in the frontal sinus. Objectives. We present a long-term follow-up of a patient with a frontal chondromyxoid fibroma who was operated four years ago. We discuss the various differential diagnoses and possible treatments. Discussion. The differential diagnosis includes chondrosarcoma, myxosarcoma, myxoma and chordoma. From the radiological point of view, chondromyxoid fibroma must be always considered as a diagnostic possibility when a solitary bone lesion appears with expansion or cortical osteolysis, lobulated margins and

  3. Prospects of the standardized treatment and research of neoplasms%骨肿瘤规范化治疗与研究展望

    Institute of Scientific and Technical Information of China (English)

    牛晓辉; 刘巍峰


    With the introduction of chemotherapy, the improvement of surgical techniques and the advocation of comprehensive and standardized treatment, great progresses have been made in the treatment of bone and soft tissue tumors. Comprehensive treatment strategy is an ideal choice for common primary bone and soft tissue tumors with surgery as the main therapy and chemoradiotherapy as the adjuvant therapy. The standardized treatment is regarded as the best strategy. The histological reaction in the neoadjuvant chemotherapy is taken as the principal method to predict the survival rate. For the patients with osteosarcoma, surgery and chemotherapy are included in the standardized treatment. It is an unclear concept of“unresectable tumors”. The tumor that can be resected by a surgeon may be unresectable for another, and it depends on how much the patient could accept the adverse effects on his or her functions. The prognosis could be predicted by the high expressions of the interferonα/β. How many metastatic lesions are there? Is it possible to resect all these lesions completely? All the factors may affect the prognosis. The wide resection combined with the preoperative chemotherapy is recommended in the treatment of resectable osteosarcoma in its progressive stage. As to the patients with unresectable metastatic tumors, the chemotherapy is a good choice. The primary tumors are evaluated, and then a suitable approach is chosen to control the local condition. The radiotherapy is the main treatment method for the patients with tumors that could not be resected by surgery. The dedifferentiated chordoma is treated according to the National Comprehensive Cancer Network ( NCCN ) guideline. It is better for the patients with metastatic giant cell tumors to undergo the treatment of intracapsular resection. Non-surgical treatment or radiotherapy is recommended for the patients with unresectable tumors. The radiotherapy can be taken as an adjutant therapy in the comprehensive

  4. Use of recombinant human bone morphogenetic protein-2 as an adjunct for instrumented posterior arthrodesis in the occipital cervical region: An analysis of safety, efficacy, and dosing

    Directory of Open Access Journals (Sweden)

    D Kojo Hamilton


    Full Text Available Background: There have been few reports on the use of recombinant human bone morphogenetic protein (rhBMP-2 in posterior spine. However, no study has investigated the dosing, safety, and efficacy of its use in the posterior atlantoaxial, and/or craniovertebral junction. Recent case report of the cytokine-mediated inflammatory reaction, following off label use of rhBMP-2 as an adjunct for cervical fusion, particularly in complex cases, has increased concern about complications associated with the product. Objective: To assess the safety, efficacy, and dosing of rhBMP-2 as an adjunct for instrumented posterior atlantoaxial and/or craniovertebral junction arthrodesis. Materials and Methods: We included all patients treated by the senior author that included posterior atlantoaxial and/or craniovertebral junction instrumented fusion using rhBMP-2 from 2003 to 2008 with a minimum two year follow-up. Diagnosis, levels fused, rhBMP-2 dose, complications, and fusion were assessed. Results: Twenty three patients with a mean age of 60.9 years (range 4 - 89 years and an average follow-up of 45 months (range 27 to 84 months met inclusion criteria. The indications for surgery included, atlantoaxial instability (n = 16, basilar invagination (n = 6, and kyphoscoliosis (n = 1. The specific pathologic diagnosis included type 2 dens fracture (n = 7, complex C1 and C2 ring fracture (n = 2, chordoma (n = 2, degenerative/osteoporosis (n = 3, rheumatoid disease (n = 8, and pseudogout (n = 1. The average rhBMP-2 dose was 2.38 mg/level, with a total of 76 levels treated (average 3.3 levels, SD= 1.4 levels. There were no complications. During the most recent follow-up, all patients had achieved fusion. Conclusions: In a series of patients with complex pathology and/or rheumatoid arthritis, 100% fusion rate was achieved with adjunct use of rhBMP-2, with a safe and effective average rhBMP-2 dose of 2.38 mg per level.

  5. The presence of local mesenchymal progenitor cells in human degenerated intervertebral discs and possibilities to influence these in vitro: a descriptive study in humans. (United States)

    Brisby, Helena; Papadimitriou, Nikolaos; Brantsing, Camilla; Bergh, Peter; Lindahl, Anders; Barreto Henriksson, Helena


    Low back pain is common and degenerated discs (DDs) are believed to be a major cause. In non-degenerated intervertebral discs (IVDs) presence of stem/progenitor cells was recently reported in different mammals (rabbit, rat, pig). Understanding processes of disc degeneration and regenerative mechanisms within DDs is important. The aim of the study was to examine the presence of local stem/progenitor cells in human DDs and if these cell populations could respond to paracrine stimulation in vitro. Tissue biopsies from the IVD region (L3-S1) were collected from 15 patients, age 34-69 years, undergoing surgery (spinal fusion) and mesenchymal stem cells (MSCs) (iliac crest) from 2 donors. Non-DD cells were collected from 1 donor (scoliosis) and chordoma tissue was obtained from (positive control, stem cell markers) 2 donors. The IVD biopsies were investigated for gene and protein expression of: OCT3/4, CD105, CD90, STRO-1, and NOTCH1. DD cell cultures (pellet mass) were performed with conditioned media from MSCs and non-degenerated IVD cells. Pellets were investigated after 7, 14, 28 days for the same stem cell markers as above. Gene expression of OCT3/4 and STRO-1 was detected in 13/15 patient samples, CD105 in 14/15 samples, and CD90 and NOTCH1 were detected 15/15 samples. Immunohistochemistry analysis supported findings on the protein level, in cells sparsely distributed in DDs tissues. DDs cell cultures displayed more undifferentiated appearance with increased expression of CD105, CD90, STRO-1, OCT3/4, NOTCH1, and JAGGED1, which was observed when cultured in conditioned cell culture media from MSCs compared to cell cultures cultured with conditioned media from non-DD cells. Expression of OCT3/4 (multipotency marker) and NOTCH1 (regulator of cell fate), MSC-markers, CD105, CD90, and STRO-1, indicate that primitive cell populations are present within DDs. Furthermore, the possibility to influence cells from DDs by paracrine signaling /soluble factors from MSCs and from

  6. SU-E-P-06: A Novel Hybrid Planning Approach to Allow More Patients Benefited by the Intensity Modulated Proton Therapy

    Energy Technology Data Exchange (ETDEWEB)

    Jiang, S [UT MD Anderson Cancer Center, Tianjin Medical University Cancer Instit, Houston, TX (United States); Liao, L [UT MD Anderson Cancer Center, University of Houston, Houston, TX (United States); Li, Y [Varian Medical Systems, Houston, TX (United States); Wang, X; Sahoo, N; Liao, Z; Grosshans, D; Frank, S [MD Anderson Cancer Center, Houston, TX (United States); Li, H [M.D. Anderson Cancer Center, Houston, TX (United States); Zhu, X; Chang, J; Zhang, X [UT MD Anderson Cancer Center, Houston, TX (United States); Gillin, M [MD Anderson Cancer Ctr., Houston, TX (United States); Hojo, Y [UT MD Anderson cancer center, Houston, TX (United States); Sun, J [UT MD Anderson cancer center, Tianjin Medical University Cancer Institute a, Houston, TX (United States)


    Purpose: We report a hybrid scattering and scanning beam delivery approach, termed as (HimpsPT), which demonstrated that majority IMPT delivery can be potentially replaced with hybrid IMPT and PSPT delivery with similar or better plan quality. Methods: Three representative clinical cases, including head and neck (HN), skull base chordoma (CNS) and lung cancer, treated in MDACC Proton Therapy Center with IMPT were retrospectively redesigned using HimpsPT. The PSPT plans are designed with the same prescriptions as those of IMPT plans. The whole treatment can be delivered by either alternating or sequential PSPT and IMPT delivery. The dosimetric data and dose distributions of HimpsPT plans are compared with those of IMPT plans. We also performed a worst-case robust analysis for all plans. Results: The target coverages for all cases are comparable. For the HN case, the mean dose of esophagus larynx, left parotid and right submandibular, oral cavity V20, the max dose of cord is 18.0, 36.1, 23.6, 47.2, 0.1, 31.7 Gy in HimpsPT plan, and 25.5, 33.8, 24.9, 49.1, 0.2, 33.8 Gy in IMPT plan. For the lung case, the lung V5, V20, V30, mean lung dose, heart V40, esophagus V70, cord maximum dose are 50.5%, 37.0%, 31.7%, 21.3 Gy, 7.2%, 4.9%, 35.5 Gy in HimpsPT plan, and 55.4%, 36.7%, 30.1%, 21.3 Gy, 7.7%, 8.4%, 36.8Gy in IMPT plans. For the CNS case, brainstem maximum dose is 50.5 Gy in HimpsPT plan and 55.4 Gy in IMPT plan due to sharp penumbra offered by the aperture of the PSPT plan in HimpsPT technique. Conclusion: For majority disease sites, the dosimetric advantage of IMPT technique can be achieved by the hybrid PSPT and IMPT technique, which enables the centers equipped with both scattering and scanning beam facilities to treat more patients which can be benefited by the scanning beam.


    Directory of Open Access Journals (Sweden)

    Ananth G


    Full Text Available BACKGROUND The endoscopic endonasal approach for the sellar region lesions is a novel technique and an effective surgical option. The evidence thus far has been conflicting with reports in favour and against a learning curve. We attempt to determine the learning curve associated with this approach. METHODS Retrospective and prospective data of the patients who were surgically treated for sellar region lesions between the year 2013 and 2016 was collected, 32 patients were operated by the endoscopic endonasal approach at Vydehi Institute of Medical Sciences and Research Centre, Bangalore. Age, sex, presenting symptoms, length of hospital stay, surgical approach, type of dissection, duration of surgery, sellar floor repair, intraoperative and postoperative complications were noted. All the procedures were performed by a single neurosurgeon. RESULTS A total of 32 patients were operated amongst which 21 patients were non-functioning pituitary adenomas, 2 were growth hormone secreting functional adenomas, 1 was an invasive pituitary adenoma, 4 were craniopharyngiomas, 2 were meningiomas, 1 was Rathke’s cleft cyst and 1 was a clival chordoma. Headache was the mode of presentation in 12 patients, 12 patients had visual deficits, 6 patients presented with hormonal disturbances amongst which 4 patients presented with features of panhypopituitarism and 2 with acromegaly. Amongst the 4 patients with panhypopituitarism, 2 also had DI, two patients presented with CSF rhinorrhoea. There was a 100% improvement in the patients who presented with visual symptoms. Gross total resection was achieved in all 4 cases of craniopharyngiomas and 13 cases of pituitary adenomas. Postoperative CSF leak was seen in 4 patients who underwent re-exploration and sellar floor repair, 9 patients had postoperative Diabetes Insipidus (DI which was transient, the incidence of DI reduced towards the end of the study. There was a 25% decrease in the operating time towards the end of

  8. Correction of patient positioning errors based on in-line cone beam CTs: clinical implementation and first experiences

    Directory of Open Access Journals (Sweden)

    Häring Peter


    Full Text Available Abstract Background The purpose of the study was the clinical implementation of a kV cone beam CT (CBCT for setup correction in radiotherapy. Patients and methods For evaluation of the setup correction workflow, six tumor patients (lung cancer, sacral chordoma, head-and-neck and paraspinal tumor, and two prostate cancer patients were selected. All patients were treated with fractionated stereotactic radiotherapy, five of them with intensity modulated radiotherapy (IMRT. For patient fixation, a scotch cast body frame or a vacuum pillow, each in combination with a scotch cast head mask, were used. The imaging equipment, consisting of an x-ray tube and a flat panel imager (FPI, was attached to a Siemens linear accelerator according to the in-line approach, i.e. with the imaging beam mounted opposite to the treatment beam sharing the same isocenter. For dose delivery, the treatment beam has to traverse the FPI which is mounted in the accessory tray below the multi-leaf collimator. For each patient, a predefined number of imaging projections over a range of at least 200 degrees were acquired. The fast reconstruction of the 3D-CBCT dataset was done with an implementation of the Feldkamp-David-Kress (FDK algorithm. For the registration of the treatment planning CT with the acquired CBCT, an automatic mutual information matcher and manual matching was used. Results and discussion Bony landmarks were easily detected and the table shifts for correction of setup deviations could be automatically calculated in all cases. The image quality was sufficient for a visual comparison of the desired target point with the isocenter visible on the CBCT. Soft tissue contrast was problematic for the prostate of an obese patient, but good in the lung tumor case. The detected maximum setup deviation was 3 mm for patients fixated with the body frame, and 6 mm for patients positioned in the vacuum pillow. Using an action level of 2 mm translational error, a target point

  9. Helical tomotherapy. Experiences of the first 150 patients in Heidelberg

    Energy Technology Data Exchange (ETDEWEB)

    Sterzing, F.; Schubert, K.; Sroka-Perez, G.; Kalz, J.; Debus, J.; Herfarth, K. [Dept. of Radiation Oncology, Univ. of Heidelberg (Germany)


    Background and purpose: helical tomotherapy was introduced into clinical routine at the Department of Radiation Oncology, University Hospital of Heidelberg, Germany, in July 2006. This report is intended to describe the experience with the first 150 patients treated with helical tomotherapy. Patient selection, time effort, handling of daily image guidance with megavoltage (MV) CT, and quality of radiation plans shall be assessed. Patients and methods: between July 2006 and May 2007, 150 patients were treated with helical tomotherapy in the University Hospital of Heidelberg. Mean age was 60 years with a minimum of 30 years and a maximum of 85 years. 79 of these patients received radiotherapy as a part of multimodal treatment pre- or postoperatively, 17 patients received treatment as a combined radiochemotherapy. 76% were treated with curative intent. Radiotherapy sites were central nervous system (n = 7), head and neck (n = 28), thoracic (n = 37), abdominal (n = 58) and skeletal system (n = 20). Most common tumor entities were prostate cancer (n = 28), breast cancer (n = 17), gastrointestinal tumors (n = 19), pharyngeal carcinoma (n = 14), lymphoma (n = 13), metastatic disease (bone n = 14, liver n = 6, lung n = 4, lymph node n = 2), sarcoma (n = 8), malignant pleural mesothelioma (n = 5), ovarian cancer treated with whole abdominal irradiation (n = 4), lung cancer (n = 3), skin malignancies (n = 3), chordoma (n = 2), meningioma (n = 2), one ependymoma and one medulloblastoma treated with craniospinal axis irradiation (n = 2), and others (n = 4). Nine patients were treated with single-fraction radiosurgery, nine with image-guided spinal reirradiation, and twelve patients were treated at multiple targets simultaneously. A pretreatment MV-CT scan was performed in 98.2% of the 3,026 fractions applied. After matching with the kilovoltage planning CT, corrections for translations and rotation around longitudinal axis (roll) were done. Results: mean time on table was 24

  10. The 100 most-cited articles in spinal oncology. (United States)

    De la Garza-Ramos, Rafael; Benvenutti-Regato, Mario; Caro-Osorio, Enrique


    OBJECTIVE The authors' objective was to identify the 100 most-cited research articles in the field of spinal oncology. METHODS The Thomson Reuters Web of Science service was queried for the years 1864-2015 without language restrictions. Articles were sorted in descending order of the number of times they were cited by other studies, and all titles and abstracts were screened to identify the research areas of the top 100 articles. Levels of evidence were assigned on the basis of the North American Spine Society criteria. RESULTS The authors identified the 100 most-cited articles in spinal oncology, which collectively had been cited 20,771 times at the time of this writing. The oldest article on this top 100 list had been published in 1931, and the most recent in 2008; the most prolific decade was the 1990s, with 34 articles on this list having been published during that period. There were 4 studies with Level I evidence, 3 with Level II evidence, 9 with Level III evidence, 70 with Level IV evidence, and 2 with Level V evidence; levels of evidence were not assigned to 12 studies because they were not on therapeutic, prognostic, or diagnostic topics. Thirty-one unique journals contributed to the 100 articles, with the Journal of Neurosurgery contributing most of the articles (n = 25). The specialties covered included neurosurgery, orthopedic surgery, neurology, radiation oncology, and pathology. Sixty-seven articles reported clinical outcomes. The most common country of article origin was the United States (n = 62), followed by Canada (n = 8) and France (n = 7). The most common topics were spinal metastases (n = 35), intramedullary tumors (n = 18), chordoma (n = 17), intradural tumors (n = 7), vertebroplasty/kyphoplasty (n = 7), primary bone tumors (n = 6), and others (n = 10). One researcher had authored 6 studies on the top 100 list, and 7 authors had 3 studies each on this list. CONCLUSIONS This study identified the 100 most-cited research articles in the area of

  11. Magnetic resonance imaging-based diagnosis of middle cranial fossa tumors%中颅窝区脑外肿瘤的MRI表现

    Institute of Scientific and Technical Information of China (English)

    段刚; 彭旭红


    Objective To analyze the magnetic resonance imaging (MRI) features of middle cranial fossa tumors. Methods Thirty-one patients with surgically and pathologically confirmed middle cranial fussa tumors were enrolled in this study, including 13 with trigeminal neurinornas, 6 with meningiomas, 3 with arachnoid cysts, 3 with dermoid cysts, 2 with osteoblastomas, 2 with chordomas, and 2 with epidermoid cysts. All the patients underwent plain and enhanced MR scanning and the imaging data were retrospectively analyzed. Results Different types of middle cranial fossa tumors had distinctly different MRI appearances. Trigeminal neuromas displayed hypo- and iso-intense signals on T1-weighted images (T1WI) and hyperintense signals on T2-weighted images (T2WI), and 9 of the 13 patients presented with shell-shaped enhancement after Gd-DTPA injection. Meningioma showed hypo-and iso-intense signals on T1WI and hypo-, iso- and hyper-intense signals on T2WI, with relatively homogeneous enhancement after Gd-DTPA injection. Osteoblastoma exhibited shell-shaped bony encapsulation of the tumor, and chordomas grew along the middle and posterior cranial fussae with close lateral association with the clivus. Arachnoid cysts and epidermoid cysts showed hypointense signals on TIWI and hyperintense signals on T2WI with slight enhancement of the cyst wall after Gd-DTPA injection. The arachnoid cysts grew expansively, and epidermoid cysts were characterized by high invasiveness in any possible directions. Dermoid cysts presented with hyperintense signals on T1WI and T2WI with popcorn-like appearance in some cases. Conclusion The middle fossa tumors present characteristic MRI features, which, in combination with clinical examinations, help derive an accurate diagnosis.%目的 探讨中颅窝区脑外肿瘤的MRI特点,提高其诊断准确率. 方法 经手术病理证实的31例中颅窝区脑外肿瘤(包括三叉神经鞘瘤13例,脑膜瘤6例,蛛网膜囊肿3例,皮样囊肿3

  12. Trombose venosa profunda e sua relação com trombofilias e neoplasias: estudo retrospectivo Deep venous thrombosis and its relationship with thrombophilias and malignancies: retrospective study

    Directory of Open Access Journals (Sweden)

    Ana Vieira Baptista


    through the review of the clinical files of all patients aged 50 or less hospitalized in our Department between January 1st 2006 and December 31st 2010 with that diagnosis, and the results obtained in the protocols were analysed. Results: From a total of 89 patients, 64 (71,9% were females and 25 (28,1% were males, and the mean age was 33,3 ±10,0 years. 14,6% of the patients had already had, at least, one previous episode of deep venous thrombosis and 78,7% had, at least, one risk factor. The protocol for screening for thrombophilia and malignancy was applied to 65 patients (73,0%, being detected congenital thrombophilias in 41 patients (63,1%, two cases of antiphospholipid antibody syndrome (APS, one case of monoclonal gammopathy, one of lung cancer and one of sacral chordoma. 49 of the patients subjected to the protocols (75,4% were referred to the Hematology Clinic for evaluation and in 40 of those patients (81,6% was suggested keeping oral anticoagulation for more than 12 months. Conclusions: Given the high prevalence of genetic polimorphisms that confer a higher thrombotic risk and the not rare cases of APS and of occult malignancies present in this population, it is considered that, despiste its elevated costs, the screening for these kind of pathologies should continue to be done.

  13. Translocación del tercio medio facial en un paciente en edad infantil: Fijación con un nuevo sistema de placas y pines reabsorbibles Mid-facial translocation in children using a new system of absorbable plates and pins: A case report

    Directory of Open Access Journals (Sweden)

    Manel Coll-Anglada


    morbidity of the procedure. Absorbable osteosynthesis material has been marketed since the 1980s as a solution to this problem. We report the case of a 13-year-old female patient diagnosed of chordoma of the clivus. A bilateral mid-facial approach was used with a new system of absorbable plates and pins affixed ultrasonically (SonicWeld®. KLS Martin, LP, Jacksonville, Florida, USA. The primary approaches to tumors located in the deep facial regions and skull base, the main variations of the mid-facial translocation technique, application of the new SonicWeld® absorbable system, and the main differences compared to traditional absorbable plates and screws are reviewed.

  14. 枕骨大孔区肿瘤MRI诊断(附52例分析)%MR imaging diagnosis of the tumors in the region of foramen magnum: analysis of 52 cases

    Institute of Scientific and Technical Information of China (English)

    葛永强; 赵清州; 李振强; 张战利


    Objective To study the clinical features and MRI appearances of the tumors in the region of foramen magnum.Methods The clinical and MRI findings of 52 patients with tumors in the foramen magnum by pathologically confirmed were analyzed retrospectively with literatures review.Results Ependymoma(9 cases),occurring mainly after the age of 40,there was cystic change,the real part of the enhanced significantly,cystic partially enhanced,state clearly.Meningioma(10 cases),wide base and enhance significant relatively homogeneous enhancement and dural tail sign there.Hemangioblastomas (7 cases),more common in young adults,showed a large cystic nodules,increased mural nodules was significantly enhanced,cystic partially characterized enhancement.Medulloblastoma (8 cases),almost were located in the cerebellum,the signal was uniform,and strengthen significant,DWI high signal.Chordoma(6 cases),and more back in the saddle,the signal uneven and bone destruction.Nerve sheath tumor(12 cases),and always traveling along the nerve with marked enhancement.Conclusions MRI imagining of the tumors in the region of foramen magnum have certain charactteristic,it has significant value in diagnosing for clinical to nature of disease.%目的 探讨枕骨大孔区肿瘤的临床和MRI诊断特点.方法 对52例经病理证实的枕骨大孔区肿瘤患者的临床和MRI表现进行回顾性分析,并复习相关文献.结果 室管膜瘤9例,主要发生于40岁以后,有囊变,实质部分强化显著,囊性部分不强化,境界清楚;脑膜瘤10例,宽基底,增强有明显相对均一强化,且有硬膜尾征;血管母细胞瘤7例,以青壮年多见,呈大囊小结节,增强壁结节明显强化,囊性部分不强化为特点;髓母细胞瘤8例,多位于小脑,信号欠均匀,强化显著,DWI为高信号;脊索瘤6例,多位于鞍背,信号不均,并有骨质破坏;神经鞘瘤12例,多沿神经走行,强化明显.结论 枕骨大孔区各种肿瘤MRI特点具有一定特征

  15. Assessment of potential advantages of relevant ions for particle therapy: A model based study

    Energy Technology Data Exchange (ETDEWEB)

    Grün, Rebecca, E-mail: [Department of Biophysics, GSI Helmholtzzentrum für Schwerionenforschung, Darmstadt 64291 (Germany); Institute of Medical Physics and Radiation Protection, University of Applied Sciences Gießen, Gießen 35390 (Germany); Medical Faculty of Philipps-University Marburg, Marburg 35032 (Germany); Friedrich, Thomas; Krämer, Michael; Scholz, Michael [Department of Biophysics, GSI Helmholtzzentrum für Schwerionenforschung, Darmstadt 64291 (Germany); Zink, Klemens [Institute of Medical Physics and Radiation Protection, University of Applied Sciences Gießen, Gießen 35390, Germany and Department of Radiotherapy and Radiation Oncology, University Medical Center Giessen and Marburg, Marburg 35043 (Germany); Durante, Marco [Department of Biophysics, GSI Helmholtzzentrum für Schwerionenforschung, Darmstadt 64291, Germany and Department of Condensed Matter Physics, Darmstadt University of Technology, Darmstadt 64289 (Germany); Engenhart-Cabillic, Rita [Medical Faculty of Philipps-University Marburg, Marburg 35032, Germany and Department of Radiotherapy and Radiation Oncology, University Medical Center Giessen and Marburg, Marburg 35043 (Germany)


    Purpose: Different ion types offer different physical and biological advantages for therapeutic applications. The purpose of this work is to assess the advantages of the most commonly used ions in particle therapy, i.e., carbon ({sup 12}C), helium ({sup 4}He), and protons ({sup 1}H) for different treatment scenarios. Methods: A treatment planning analysis based on idealized target geometries was performed using the treatment planning software TRiP98. For the prediction of the relative biological effectiveness (RBE) that is required for biological optimization in treatment planning the local effect model (LEM IV) was used. To compare the three ion types, the peak-to-entrance ratio (PER) was determined for the physical dose (PER{sub PHY} {sub S}), the RBE (PER{sub RBE}), and the RBE-weighted dose (PER{sub BIO}) resulting for different dose-levels, field configurations, and tissue types. Further, the dose contribution to artificial organs at risk (OAR) was assessed and a comparison of the dose distribution for the different ion types was performed for a patient with chordoma of the skull base. Results: The study showed that the advantages of the ions depend on the physical and biological properties and the interplay of both. In the case of protons, the consideration of a variable RBE instead of the clinically applied generic RBE of 1.1 indicates an advantage in terms of an increased PER{sub RBE} for the analyzed configurations. Due to the fact that protons show a somewhat better PER{sub PHY} {sub S} compared to helium and carbon ions whereas helium shows a higher PER{sub RBE} compared to protons, both protons and helium ions show a similar RBE-weighted dose distribution. Carbon ions show the largest variation of the PER{sub RBE} with tissue type and a benefit for radioresistant tumor types due to their higher LET. Furthermore, in the case of a two-field irradiation, an additional gain in terms of PER{sub BIO} is observed when using an orthogonal field configuration

  16. Surgical treatment for giant tumors of the pelvis%骨盆巨大肿瘤的手术治疗

    Institute of Scientific and Technical Information of China (English)

    齐波; 丁晶; 徐永清; 阮默; 林月秋


    Objective To explore the method of surgical treatment for giant tumors of the pelvis. Methods 21 cases with giant tumors of the pelvis were hospitalized in the period from Jan. ,2000 to Dec. ,2007,17 male and 4 female,the average age was 55 years old(from 35 to 65),including 8 cases of sacral chordoma,5 cases of iliac chondrosarcoma,4 cases of chondroblastoma,3 cases of giant cell tumor(GCT)of bone and 1 case of osteosarcomas;all the patients underwent surgical resection with temporary blocking of bilateral or unilateral common iliac arteries. Results Follow-up lasted from 0. 5 year to 6 years with the average of 4 years;local reoccurrence was found in 4 cases of sacral chordom,1 case died of lung metastases and systemic failure,1 case died of lung metastases 1. 5 year after surgery;no reoccurrence was found in other cases;8 cases had discomfort with pain of unknown causes in the operation sites. Conclusions Temporary blocking of bilateral or unilateral common iliac artery in operation can obviously reduce the bleeding, make a complete resection of the tumors at most and maintain the stability of the pelvis,which raises the surgical effect on patients with giant tumors of pelvis.%目的:探讨骨盆巨大肿瘤的手术治疗方法。方法我院自2000年1月~2007年12月共收治21例骨盆巨大肿瘤患者,男17例,女4例,平均55(35~65)岁。其中骶骨脊索瘤8例,髂骨软骨肉瘤5例,软骨母细胞瘤4例,骨巨细胞瘤3例,骨肉瘤1例,均采用双侧或单侧髂总动脉暂时阻断的方法行手术切除。结果所有患者术后获得0.5~6年(平均4年)随访,脊索瘤4例局部复发,1例死于肺部转移全身衰竭,1例于术后1年半死于肺部转移,其余无复发;8例出现手术部位不明原因疼痛不适。结论术中采用双侧或单侧髂总动脉暂时阻断的方法可明显减少术中出血,尽量彻底切除瘤体及维持骨盆稳定性等,可明显提高骨盆巨大肿瘤患者的手术疗效。

  17. Proton therapy - Present and future. (United States)

    Mohan, Radhe; Grosshans, David


    In principle, proton therapy offers a substantial clinical advantage over conventional photon therapy. This is because of the unique depth-dose characteristics of protons, which can be exploited to achieve significant reductions in normal tissue doses proximal and distal to the target volume. These may, in turn, allow escalation of tumor doses and greater sparing of normal tissues, thus potentially improving local control and survival while at the same time reducing toxicity and improving quality of life. Protons, accelerated to therapeutic energies ranging from 70 to 250MeV, typically with a cyclotron or a synchrotron, are transported to the treatment room where they enter the treatment head mounted on a rotating gantry. The initial thin beams of protons are spread laterally and longitudinally and shaped appropriately to deliver treatments. Spreading and shaping can be achieved by electro-mechanical means to treat the patients with "passively-scattered proton therapy" (PSPT) or using magnetic scanning of thin "beamlets" of protons of a sequence of initial energies. The latter technique can be used to treat patients with optimized intensity modulated proton therapy (IMPT), the most powerful proton modality. Despite the high potential of proton therapy, the clinical evidence supporting the broad use of protons is mixed. It is generally acknowledged that proton therapy is safe, effective and recommended for many types of pediatric cancers, ocular melanomas, chordomas and chondrosarcomas. Although promising results have been and continue to be reported for many other types of cancers, they are based on small studies. Considering the high cost of establishing and operating proton therapy centers, questions have been raised about their cost effectiveness. General consensus is that there is a need to conduct randomized trials and/or collect outcomes data in multi-institutional registries to unequivocally demonstrate the advantage of protons. Treatment planning and plan

  18. 孤立性蝶窦疾病159例临床分析%Isolated sphenoid sinus disease: a clinical analysis of 159 cases

    Institute of Scientific and Technical Information of China (English)

    肖喜艳; 巴云鹏; 张玉杰; 魏燕高


    目的:分析孤立性蝶窦疾病的诊断与治疗方法,使其得到早期诊断、及时干预和治疗,以降低误诊率.方法:对159例孤立性蝶窦疾病患者进行回顾性分析.结果:孤立性蝶窦疾病患者最常见的临床症状是头痛(79.87%,127/159).在159例患者中,60例(37.74%)为蝶窦囊肿,44例(27.67%)为单纯性蝶窦炎,31例(19.50%)为真菌性蝶窦炎,5例(3.14%)为蝶窦息肉,3例(1.89%)为骨纤维异常增殖症,2例(1.26%)为内翻性乳头状瘤,3例(1.89%)为脊索瘤,3例(1.89%)为鳞状细胞癌,3例(1.89%)为恶性淋巴瘤,2例(1.26%)为神经内分泌癌,2例(1.26%)为嗅神经母细胞瘤,1例(0.63%)为恶性纤维组织细胞瘤.术后随访10个月~4年,鼻内镜术后多数患者转归良好.结论:孤立性蝶窦疾病常无特异性症状及体征,最常见的临床症状是头痛,其次是视力障碍,鼻内镜下蝶窦开放术是首选的治疗方法.%Objective: Aimed to observe and analyse the diagnostic and therapeutic methods of the isolated sphenoid sinus disease, achieve earlier diagnosis and timelier intervention for this easily neglected disease and decrease the occurrence of misdiagnoses. Method: A retrospective study was conducted in 159 patients with isolated sphenoid sinus disease. Result: Headache was the most common presenting symptom(79. 87%, 127/159). Among the 159 cases, 60(37. 74%)had mucocele, 44(27. 67%) isolated sphenoiditis, 31(19. 50%) fungal sinusitis, 5 (3.14%) polyp, 3(1.89%) fibrous dysplasia, 2(1.26%) inverted papilloma, 3(1.89%) chordoma, 3 (1.89%) squamous carcinoma, 3(1. 89%) malignant lymphoma, 2(1. 26%) neuroendocrine carcinoma, 2 (1. 26%) olfactory neuroblastoma, and 1(0. 63%) malignant fibrohistiocytoma. A follow-up of 10 months to 4 years post-surgery showed good prognosis in most of the patients who underwent surgical therapy. Conclusion:The sphenoid sinus disease is often vague and nonspecific in its clinical presentation. The most common

  19. CT and MRI manifestations of sacrum tumors involving sacroiliac joint%骶骨肿瘤侵犯骶髂关节的CT及MRI表现

    Institute of Scientific and Technical Information of China (English)

    娄路馨; 程晓光; 白荣杰; 张薇; 梁伟; 张卉


    目的 分析良、恶性骶骨肿瘤侵犯骶髂关节的CT及MRI表现.方法 观察51例骶骨肿瘤对骶髂关节侵犯的CT和MRI表现.其中骨巨细胞瘤19例,神经鞘瘤6例,神经纤维瘤1例,脊索瘤7例,恶性纤维组织细胞瘤、小细胞肉瘤和梭形细胞肉瘤各1例,软骨肉瘤6例,尤因肉瘤2例,黏液乳头状室管膜瘤1例,转移癌6例.结果 骶髂关节受侵29例(29/51,56.86%),其中双侧骶髂关节受侵11例,单侧受侵18例.骶髂关节受侵发生率在良性肿瘤患者中为53.85%(14/26),在恶性患者中为60.00%(15/25).CT表现为关节双侧骨质破坏、关节面消失,软组织肿块占据骶髂关节;MRI表现为骶髂关节双侧骨质信号消失,被肿瘤信号取代.结论骶骨恶性肿瘤常侵犯骶髂关节.骶骨良性肿瘤中,骨巨细胞瘤最易侵犯骶髂关节.%Objective To observe CT and MRI manifestations of the invasion of sacroiliac joint by benign and malignant sacrum tumor.Methods CT and MR images of 51 patients with the invasion of sacroiliac joint by sacrum tumors, including 19 giant cell tumors, 6 schwannomas, 1 neurofibroma, 7 chordomas, 1 malignant fibrohistiocytoma, 1 small cell sarcoma, 1 spindle cell sarcoma, 6 chondrosarcomas, 2 Ewing sarcomas, 1 myxopapillary ependymoma and 6 metastases were analyzed.Results Sacroiliac joint invasion was found in 29 patients (29/51, 56.86%).Bilateral invasion of sacroiliac joint occurred in 11, while 18 unilateral invasion occurred in 18 cases.The rate of invasion of sacroiliac joint was 53.85 % (14/26) in patients with benign tumors and 60.00% (15/25) in patients with malignant tumors.Bilateral bone destruction of the joints, disappearance of articular surface and the replacement of sacroiliac joint by soft tissue mass were observed on CT.MRI showed that the signal of bilateral sacroiliac joint were replaced by tumor signal.Conclusion Sacroiliac joint is easily to be invaded by malignant sacrum tumors, as well as by giant cell tumor.

  20. Surgical reconstruction strategy of high level sacral tumors after tumor resection%高位骶骨肿瘤切除后的外科重建策略

    Institute of Scientific and Technical Information of China (English)

    吴强; 邵增务; 杨述华; 王佰川; 范磊


    目的探讨高位骶骨肿瘤切除后个性化重建方案。方法回顾分析自2000年9月至2011年12月,手术治疗的高位骶骨肿瘤11例,肿瘤类型包括骨巨细胞瘤、脊索瘤、软骨肉瘤、骨肉瘤及神经源性肿瘤。所有病例中,骶椎S1均受累。根据骶骨及骶髂关节侵犯范围,选择最佳手术方案,进行个性化外科重建。结果全部11例无术中死亡病例,术中平均出血量3200 ml。10例获得8个月至6年的随访,平均24个月,术后近期并发症1例为切口皮缘坏死和伤口延迟愈合;1例术后出现排尿困难,1例脑脊液漏。患者早期功能恢复良好,神经功能障碍改善率达66.7%。局部复发2例,分别为1例骨巨细胞瘤和1例软骨肉瘤,未出现远处转移病例。随访病例均未发现钉棒松动、断裂,以及同种异体腓骨植入后骨端吸收现象。结论良好的手术计划以及个性化的切除及重建方案可以保证手术的成功性。减少术中出血、合适地保留马尾神经功能以及骨盆环的重建是手术考虑的重点。%Objective The surgery of high level sacrum is a challenge in the ifeld of bone tumor therapy because of its special anatomic structure, large quantity of hemorrhage during the operation and the difficulty in reconstruction. This study is to investigate the individual reconstruction strategy of high level sacral tumor surgery. Methods The retrospective study included 11 patients from September 2000 to December 2011. The tumor type included bone giant cell tumor, chordoma, chondrosarcoma, osteosarcoma and neurogenic tumor. In all cases, the sacral vertebrae S1 was involved. The individualized reconstruction strategy was conducted according to the invasion area of the sacrum and sacroiliac joint. Results No patients died during the operation. The average amount of bleeding was 3200 ml. 10 cases were followed up for 8 months to 6 years, 24 months in average. The recent

  1. 微囊性/网状神经鞘瘤的临床病理特点%Microcystic/reticular schwannoma occurring in cervical spine: report of a case with literature review

    Institute of Scientific and Technical Information of China (English)

    李百周; 王加伟; 魏红权


    Objective To study the morphologic characteristics, immunophenotype and differential diagnosis of a case of microcystic/reticular schwannoma occurring in cervical spine. Methods The pathologic features and immunophenotypic profile of a case of microcystic/reticular schwannoma were studied. Immunohistochemistry was performed using EnVision two-step method. Results The patient was a 35 -year-old male and presented with a bump over the fifth cervical spine on radiologic check up. Crossly,the bump was gray-white in color, soft, well-circumscribed but non-encapsulated. The tumor measured 3.5 cm ×3.0 cm × 1. 8 cm in size. Histologically, it was composed of two distinctive components. One component resembled the conventional schwannoma but showed focally nuclear pleomorphism, reminiscent of changes in degenerating schwannoma. The other component consisted of epithelial-like cells arranged in a reticular or lacelike pattern, amongst a myxoid matrix. Immunohistochemical study showed that the tumor cells were strongly positive for vimentin, S-100 protein, glial fibrillary acidic protein and neuron-specific enolase, focally positive for CD68, CD10 and Ki-67, and negative for pan-cytokeratin, epithelial membrane antigen, neurofilament, carcinoembryonic antigen, smooth muscle actin, estrogen receptor, progesterone receptor and p53. Conclusions Microcystic/reticular schwannoma is a novel variant of schwannoma,arising mainly in internal viscera but seldom in bone. Awareness of this entity is helpful in distinction from chordoma, other mucoid tumors or sarcomas.%目的 探讨微囊性/网状神经鞘瘤的形态学和免疫表型特点及鉴别诊断.方法 收集1例发生在颈椎的微囊性/网状神经鞘瘤,根据HE切片和免疫组织化学染色观察形态学及免疫表型特点,并复习相关文献.免疫组织化学采用EnVision两步法.结果 患者男,35岁,因颈部不适就诊,影像学检查见颈5椎体破坏,肿块周围有硬化带,考虑良性病变.手

  2. The surgical treatment of musculo- skeletal tumors in dangerous region%危险区骨骼肌肉系统肿瘤的外科治疗

    Institute of Scientific and Technical Information of China (English)

    张杏泉; 李开华; 陈刚; 刘军; 蔡平; 方仁义; 王民政; 范清宇


    目的:介绍一种安全处理危险区骨骼肌肉系统良性或低度恶性肿瘤的外科治疗方法.方法:对骶2脊索瘤先施行前路经腹结扎双侧髂内动脉,然后再切除肿瘤,可减少出血.对腹股沟区或腘窝区较大肿瘤与股、腘大血管及神经紧密粘连者,先从肿瘤远近端血管、神经正常部分解剖出血管神经,再向病变区解剖,容易完整切除肿瘤.对锁骨上区肿瘤,先截除一段锁骨,然后从肿瘤远近端正常锁骨下动、静脉及臂丛神经处,向肿瘤部游离并保护好胸膜,可较安全地切除肿瘤.而对于腓骨头、颈及其周围部肿瘤,先解剖出腓总神经及其各肌支,尽可能保留肌支.结果:11例危险区(紧邻大血管、神经区域)良性或低度恶性骨骼肌肉系统肿瘤,采用先从肿瘤远近端正常血管、神经处游离出神经、血管,再向肿瘤部解剖,均顺利解剖出大血管及神经,并完整切除肿瘤.所有病例无复发,亦无肢体功能障碍.结论:对紧邻重要血管及神经的较大良性或低度恶性骨骼肌肉系统肿瘤,先从正常段血管、神经向肿瘤部解剖游离,既可彻底切除肿瘤,又可避免损伤血管、神经引起肢体功能障碍,是一种较好方法.%Objective:To introduce a surgical treatment of the innocent or low - degree malignant musculo - skeletal tumors in dangerous region. Methods: For the sacral 2 chordoma,to perform (he ligation of the two — side internal iliac artery first through abdominal cavity and then excise the tumors in order to reduce bleeding. For the large tumors especially adhering tightly with important nerves and vessels in inguinal region or popliteal fossa, to dissect first the normal vascular nerve of tumors distantly and proximally and then disconnect the lesion for excision of tumors completely. For the tumors in supraelavicular region, to dissect the normal vascular nerve of infraclavicula and brachial plexus of tumors distantly and proximally

  3. 骶骨肿瘤切除术辅助氩氦刀的临床疗效分析%An analysis of clinical effects of surgical resection of sacral tumors adjuvant with Cryo-Hit

    Institute of Scientific and Technical Information of China (English)

    彭智恒; 王吉兴; 江建明; 瞿东滨; 鲁凯伍; 蒋晖; 王海明; 陈建庭


    Objective To evaluate the clinical effects of surgical resection of sacral tumors adjuvant with Cryo-Hit. Methods From January 2007 to December 2012, the clinical data of 15 consecutive patients with sacral tumors who were treated with resection adjuvant with Cryo-Hit and were followed up were retrospectively analyzed. There were 9 males and 6 females, whose average age was 51.3 years old ( range;38-78 years ). There were 12 cases of chordomas, 1 case of giant cell tumor of bone, 1 case of ifbromatosis in the sacroiliac region and 1 case of metastatic tumor in the sacrum, which were conifrmed by the postoperative pathology. Tumors were found in S2 ( n=8 ) and below S3 ( n=6 ). Recurrence was noticed in the buttock in 1 case. The course ranged from 2 months to 9 years. Sacrococcygeal pain occurred in 90%of all the patients, sciatica in 6 patients, and bladder and ( or ) rectal dysfunction in varying degrees in 12 patients. All the patients received surgical resection of sacral tumors adjuvant with Cryo-Hit. A combination of anterior and posterior approaches was adopted in 14 patients. In the anterior approach isolation and exposure of tumors was performed under laparoscope, and in the posterior approach Cryo-Hit was used to freeze and completely resect tumors. Palliative posterior surgery was performed alone on 1 patient with metastatic tumor in the sacrum, due to the poor body condition. Results The mean operation time was 315 min, and the mean blood loss was 1065 ml. The mean drainage lfow was 635 ml, and the mean postoperative hospital stay was 19.2 days. All the patients were followed up for an average period of 26 months ( range;5-48 months ). There were 14 patients alive, and the total survival rate was 93.3%. The bilateral nerve roots in S2 and above were successfully spared in all the patients, and no motor or sensory disturbances of lower limbs were noticed. The Visual Analogue Scale ( VAS ) scores were ( 6.80±1.52 ) points and ( 2.33±0.90 ) points

  4. Clinical analysis on the surgical treatment of periacetabular tumors%髋臼部位肿瘤手术治疗的临床分析

    Institute of Scientific and Technical Information of China (English)

    倪建法; 周幸; 周光新; 计忠伟; 胡斌; 陆萌; 吴苏稼; 施鑫


    discuss the methods, effects, and complications of surgical treatment of periacetabular tumors. Methods We retrospectively analyzed the clinical data of 36 cases of periacetabular tumor surgically treated in our depart-ment, including 21 males and 15 females, aged 15 to 64 (45.2 ±6.3) years.Among them, there were 8 cases of benign tumor (4 ca-ses of simple bone cyst, 1 case of desmoplastic fibromas, 1 case of osteofibrous dysplasia, and 2 cases of hemangioma) and 28 cases of malignant tumor (5 cases of osteosarcomas, 12 cases of chondrosarcomas, 2 cases of giant cell tumor of the bone, 1 case of malignant chondroblastoma, 2 cases of malignant fibrohistiocytoma, and 5 cases of metastatic tumor of the bone).The benign cases received sim-ple tumor resection plus bone graft and steel plate system internal fixation, while malignant cases underwent reconstruction by combined pedicle screw fixation system with bone cement. Results No patients died perioperatively and controllable complications occurred in 5 cases after operation.All the patients were followed up for 5 to 96 months except for 1 case of benign tumor.None of the benign cases experienced recurrence, and all of them achieved normal hip function.Lung metastases occurred in 10 malignant cases (4 cases of os-teosarcomas and 6 cases of chondrosarcomas) , who died of respiratory failure, and the other 18 remained tumor-free after surgery.Of the 2 patients with malignant fibrohistiocytoma, 1 died 2 years postoperatively and the other survived with tumor.Neither local recur-rence nor lung metastasis was found in the patients with chordoma and malignant chondroblastoma till the end of follow-up.The 5 pa-tients with metastases died within 3 years after operation.At 3 months after surgery, the Harris scores after total hip replacement were >90 in the 7 benign cases, 70-79 in 9 malignant cases, and <70 in the other 19 malignant cases. Conclusion For the treatment of malig-nant tumors, reconstruction by combined pedicle screw

  5. Total sacrectomy via posterior approach for malignant sacral tumors%单纯后路全骶骨切除治疗骶骨恶性肿瘤

    Institute of Scientific and Technical Information of China (English)

    孙伟; 陈泉池; 马小军; 华莹奇; 蔡郑东


    high level (S1,S2).Methods 5 cases of primary malignant sacral tumors treated by total sacrectomy via posterior approach and iliolumbar reconstruction from March 2010 to March 2011 were analyzed retrospectively.There were 3 males and 2 females.The mean age was 41,ranging from 32 to 55.The imageology examination showed osteolytic destruction,among which there were 4 cases of obvious soft tissue mass,1 case of obvious sacral foramina expansion with bone damage.MRI showed 1 case of the tumors in S1-S5,1 case of the tumors in S1 、S2,1 case of the tumors in S1-S3.The smallest tumor was 9.2 cm×7.6 cm×4.1 cm,while the largest was 22.0 cm× 19.0 cm× 16.0 cm.Preoperative TTNB were done on the 5 patients.Pathological diagnosis:2 cases of chordoma,1 case of malignant neurilemmoma,1 case of chondrosarcoma and 1 case of malignant hemangioendothelioma.5 patients had sacrococcygeal pain or lumbocrural pain before the surgery.Visual analogue scale (VAS):2 cases of 2,2 cases of 6 and 1 case of 8.Ilium stability reconstructions were all performed on 5 patients with spine pedicle screw-rod system.Results The mean operation time was 6.5 hours (range,4.5-11 hours),with the mean intraoperative blood loss of 3 700 ml (range,2 000-7 200 ml).There was no perioperative death.The mean follow-up time was 17 months (range,9-23 months).There were 2 cases of wound complications 2 weeks after surgery and healed by second intention with washing and drainage after debridement.There was no deep infection.1 case of rectal injury,which was performed with colostomy during the operation,and stoma returned 12 weeks after surgery.4 cases of sciatic nerve symptom of lower limbs and plantar flexion dyskinesia after bilateral S1 nerve roots resection.The patients walked with ankle brace fixed after the surgery.There was 1 case of implant breakage and no obvious spine down.5 patients had functional disability in sphincter after surgery.1 case of hemangioendothelioma recurred locally 9 months later