gms | German Medical Science

Objective: Hemangioblastoma is a benign tumor of the central nervous system, which usually occurs in the cerebellum, brainstem or spinal cord. Multiple hemangioblastomas occur in von Hippel-Lindau disease (VHL). Extradural spinal nerve hemangioblastomas have only been described in a few case reports. These rare lesions harbor diagnostic and therapeutic pitfalls.

Methods: We reviewed our hemangioblastoma database for patients with extradural spinal nerve hemangioblastomas.

Results: Between 1983 and 2003 six patients were operated for spinal nerve hemangioblastomas at our institution. Radiographically, the tumors could easily be mistaken for schwannomas or metastases. However, they did have some typical features. Although the surgical outcome of these lesions is generally good, complications including profuse hemorrhage and local recurrence occur more frequently than in medullary hemangioblastomas.

Conclusions: Spinal nerve hemangioblastomas harbor diagnostic and therapeutic pitfalls. Clinically significant bleeding as well as local tumor recurrence is more common than in intradural hemangioblastomas, mostly because of erroneous initial radiographic diagnosis. The fascicle of origin should be identified and resected. The tumor should not be entered by the surgeon. Because of the surgical consequences, hemangioblastoma should always be considered as an important radiological differential diagnosis of nerve sheath tumors.