Analysis of prognostic factors in 146 patients with anterior skull base sarcoma: an international collaborative study.
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BACKGROUND: Single-institutional studies lack sufficient power to assess the role of surgery and radiotherapy in the management of sarcomas involving the anterior skull base. For this study, an international collaborative study group analyzed a large cohort of patients who underwent surgery for the treatment of skull base tumors. METHODS: A subset of 146 patients who had a histologic diagnosis of skull base sarcoma (SBS) formed the basis of this report. Most patients were aged > or =21 years (77%) and had stage IV disease (56%). Adjuvant radiotherapy was received by 35% of patients, and chemotherapy was received by 10% of patients. RESULTS: Orbital involvement was encountered in 53% of patients, involvement of the orbital wall was encountered in 46% of patients, and intracranial extension was encountered in 28% of patients. Positive microscopic margins were reported in 43% of patients (51 of 118 patients). Treatment-related complications were reported in 27% of patients, and postoperative mortality was reported in 1.4% of patients. With a median follow-up of 34 months, the 5-year overall, disease-specific, and recurrence-free survival rates were 62%, 64%, and 57%, respectively. Tumor grade and adjuvant radiotherapy were not significant predictors of survival. Prior radiotherapy, intraorbital extension, positive margins, and postoperative complications were significant predictors of reduced disease-specific survival on univariate analysis. The presence of positive/close margins, however, was the only independent predictor of poor overall, recurrence-free, and disease-specific survival on multivariate analysis (relative risk, 2.4; P = .006). The 5-year disease-specific survival rate was 77%, 43%, and 36% for patients with negative, close, and positive margins, respectively. CONCLUSIONS: The current results indicated that wide craniofacial resection with negative margins is an independent prognostic predictor of better outcome. Patients with positive margins have a high risk for tumor recurrence independent of tumor grade. (+info)
Synovial sarcoma of the sellar region.
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Primary sarcomas of the sellar region are uncommon, although a wide variety have been reported. To date, no cases of primary synovial sarcoma have been described as occurring at this site. We report an immunohistochemically and molecular genetically confirmed primary synovial sarcoma involving the sellar/parasellar region and cavernous sinus in an adult male. Subtotal resection and radiosurgery proved to be efficacious. The spectrum of primary sellar region sarcomas is summarized. (+info)
Chordoma and chondrosarcoma: similar, but quite different, skull base tumors.
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BACKGROUND: Chordoma and chondrosarcoma of the skull base are frequently amalgamated because of similar anatomic location, clinical presentation, and radiologic findings. The chondroid chordoma variant has been reported to carry a better prognosis. The objective of the current study was to investigate the distinctions between these 3 entities. METHODS: The data concerning 109 patients with chordoma, chondroid chordoma, and chondrosarcoma who were treated by a single surgeon with maximum surgical resection and frequently by adjunct proton beam radiotherapy between 1990 and 2006 were analyzed retrospectively. Pathologic distinction was established by cytokeratin and epithelial membrane antigen staining. Clinical, radiologic, pathologic, and cytogenetic studies were analyzed in relation to disease recurrence and death. RESULTS: The average follow-up was 48+/-37.5 months (range, 1-191 months). There were no reliable distinguishing clinical or radiologic features noted between the groups. Chondrosarcoma patients had a significantly better outcome compared with chordoma patients with regard to survival and recurrence-free survival (P=.028 and P<.001, respectively), whereas patients with chondroid chordoma had a poor outcome similar to chordoma patients with regard to survival and recurrence-free survival (P=.337 and P=.906, respectively). CONCLUSIONS: Chordoma and chondrosarcoma differ with regard to their origin and histology, and differ markedly with regard to outcome. Chondroid chordomas behave in a manner that is clinically similar to chordomas, with the same prognosis. Both chordoma types demonstrate an aggressive clinical course and poor outcome after disease recurrence. The optimal treatment for all groups of patients involves radical surgical resection followed by high-dose radiotherapy in patients with chordomas. Radiotherapy may not be necessary in patients with low-grade chondrosarcoma. (+info)
Removal of clival chordoma in an adolescent thorough combned pterional transsylvian and anterior temporal approach.
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Extensive and aggressive surgical removal is treatment of choice for patients who have chordomas of the cranial base. Well-developed microsurgical techniques, as well as good surgical judgment learned from experience are essential to avoid major morbidity. A 14-year-old female presented with progressive headaches and diplopia for three months. Cranial magnetic resonance imaging demonstrated a well-circumscribed mass in the clival region. The mass was totally excised via right combined pterional transsylvian and anterior temporal approach (+ orbitozygomatic osteotomy). The tumor was located extradurally. The resected tumor had the typical histological and immunohistochemical characteristics of chordoma. No radiation therapy or chemotherapy was administered. (+info)
Posterior fossa dermoid cysts in association with Klippel-Feil syndrome: report of three cases.
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Although the association of spinal lumbosacral dysraphism and congenital spinal dermoid tumors is well known, the association of craniocervical spinal anomalies and posterior fossa dermoids has only been recognized recently. Advances in imaging technology and awareness of the association likely contribute to an increase in recently reported cases. (+info)
Expression of hepatocyte growth factor and c-MET in skull base chordoma.
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BACKGROUND: Hepatocyte growth factor (HGF) is a multipotent cytokine that is mediated by its receptor, c-MET. HGF/c-MET contributes to tumor progression in many human malignancies; however, HGF/c-MET is inversely correlated with aggressive biologic behavior in other cancers. Conversely, to the authors' knowledge, little is known regarding the significance of HGF/c-MET expression in skull base chordoma. METHODS: Using immunohistochemical techniques, the authors investigated HGF/c-MET expression in 46 primary and 25 recurrent lesions, and compared it with the expression of proteinases and cell differentiation markers, proliferative ability, and other clinicopathologic parameters. RESULTS: c-MET was found to be expressed in 70.0% of primary and 88.0% of recurrent lesions. HGF expression was scarcely detected. Higher c-MET expression was found to be correlated with younger patient age. Lesions with a higher expression of low molecular weight cytokeratin (CAM5.2) demonstrated significantly higher c-MET scores in both primary and recurrent lesions compared with those with lower CAM5.2 expression. In recurrent lesions, higher c-MET expression was found to be associated with the scores of matrix metalloproteinase (MMP)-1, MMP-2, tissue inhibitor of matrix metalloproteinase-1, and urokinase plasminogen activator (uPA); however, only uPA was found to be correlated with higher c-MET expression in primary lesions. c-MET expression did not appear to be correlated with MIB-1 labeling index. Patients with higher c-MET expression were found to have longer survival. CONCLUSIONS: In the current study, c-MET expression was a common event, and was found to be correlated with CAM5.2 expression, younger patient age, and a favorable prognosis in patients with skull base chordoma. However, HGF/c-MET paracrine signaling also may contribute to its invasive ability, especially in recurrent lesions. (+info)
Maffucci's syndrome complicated by intracranial chondrosarcoma: two new illustrative cases.
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Maffucci's syndrome is a rare congenital condition, sometimes misdiagnosed as Ollier's disease, characterized by multiple enchondromas combined with hemangiomas and phlebectasia. Coexisting primary malignancies have been described sporadically. We report two cases of Maffucci's syndrome associated with cranial base chondrosarcoma, emphasizing pathophysiological features and the challenging management of intracranial chondrosarcomas. To the best of our knowledge, only twelve similar cases have been reported in the literature. (+info)
Malignant transformation of clival chordoma after gamma knife surgery - case report - .
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A 54-year-old woman presented a midline clival tumor manifesting as right abducens palsy in May 1997. Magnetic resonance (MR) imaging revealed a midline clival tumor. She underwent surgery twice with the transsphenoidal approach and gamma knife surgery for residual tumor. The histological diagnosis was chordoma. MR imaging revealed that the tumor had extended to the right cerebellopontine angle, with spinal seeding in February 2002. She underwent partial removal of the right cerebellopontine angle tumor. The histological diagnosis was chordoma with slight nuclear atypism. She died 5 years and 5 months after the first gamma knife surgery. Autopsy revealed multiple areas of spinal seeding. Histological examination confirmed malignant transformation with unique epithelial characteristics, possibly caused by gamma knife surgery. (+info)