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

ABSTRACT

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.