Cranial nerve hemangioblastomas. Report of a rare case and review of literature.

BACKGROUND: Cranial nerve hemangioblastomas are exceedingly rare lesions. We review available literature and present the first surgical report on a solid hemangioblastoma arising from the trigeminal nerve and involving the Meckel cave that was successfully treated with selective embolization and successive surgical resection. CASE DESCRIPTION: A 54-year-old woman presented with an 8-month history of facial numbness associated with headache. An MRI revealed a highly vascularized supra- and infratentorial mass involving the right Meckel cave. A preoperative angiogram with selective embolization of tumoral feeders was performed, and the patient elected to undergo surgical treatment. A large solid lesion encasing the trigeminal nerve was resected without complications via a PLPA approach. Hystopathological examination revealed features consistent with the diagnosis of hemangioblastoma. Other stigmata or familiar history of VHL disease were absent. CONCLUSIONS: Review of the literature confirms that cranial nerve hemangioblastomas are very rare lesions that occur sporadically or in association with VHL disease. Surgical management of such lesions should be directed by clinical and radiological features as well as patient expectations.

Intradural cranial chordoma: a rare presentation of an uncommon tumor. Surgical experience and review of the literature.

OBJECT: Intracranial intradural chordomas are extremely rare lesions, and only a few cases have been reported in the neurosurgical literature. The authors performed a retrospective analysis of cases treated at their institutions. They present an illustrative surgical scenario and discuss the published literature, pathogenesis, and histopathological features as well as available follow-up data on the clinical behavior of these intradural lesions. METHODS: The authors reviewed clinical, neuroimaging, operative and follow-up data from a series of 79 chordomas treated at their institutions over the last 17 years. They found that the tumors were confined exclusively to the intradural compartment in only three cases. Staining for MIB-1 was performed to support the differential diagnosis between ecchordosis physaliphora and intradural chordoma, and in all three patients the neoplastic nature of the lesions was confirmed. None of these three cases showed recurrence of the lesion at midterm follow up. CONCLUSIONS: Complete resection, followed by close clinical and neuroradiological follow up, is warranted in cases of intradural cranial chordoma.

Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chondrosarcomas.

OBJECTIVE: To evaluate patient clinical outcome and survival at long-term follow-up after aggressive microsurgical resection of chondrosarcomas of the cranial base. METHODS: Over a 20-year period, 47 patients underwent 72 operative procedures for resection of cranial base chondrosarcomas. Thirty-three patients were previously untreated, whereas 14 patients previously had undergone surgery or radiation. Twenty-three patients had a single operation and 24 underwent staged (more than one) operations because of extensive disease. Patients who underwent subtotal resection also underwent radiotherapy or radiosurgery. Patients were evaluated at follow-up clinically and by imaging studies. RESULTS: Gross total resection was accomplished in 29 (61.7%) patients, and subtotal resection was accomplished in 18 patients (38.3%). The resection was better in patients who underwent a primary operation (gross total resection, 68.8 versus 46.7%) rather than a reoperation. Patients who underwent incomplete resection underwent postoperative radiotherapy, which included proton beam radiotherapy (15.6%), radiosurgery (68%), and fractionated radiation (15.6%). There were no operative deaths. Postoperative complications (cerebrospinal fluid leakage, quadriparesis, infections, cranial nerve palsies, etc.) were observed in 10 patients (18%). The follow-up ranged from 2 to 255 months, with an average of 86 months. At the conclusion of study, 36 (76.6%) patients were alive, and 21 (44.7%) patients were alive without disease. Recurrence-free survival was 32% at 10 years in all patients, 42.3% in primary patients and 13.8% in those who underwent reoperation. The Karnofsky performance score was 82.4 +/- 9.8 before surgery, 85 +/- 12.5 at 1 year after surgery, and 85.3 +/- 5.8 at the latest follow-up. Two patients died as a result of radiotherapy complications (malignancy, radiation necrosis). CONCLUSION: Cranial base chondrosarcomas can be managed well by complete surgical resection or by a combination of surgery and radiotherapy. The study cannot comment about the efficacy of radiotherapy. Approximately half of the patients survived without recurrence at long-term follow-up (>132 mo). The functional status of the surviving patients was excellent at follow-up.