Posterior fossa hemangioblastomas

Background: Hemangioblastomas are histologically benign lesions that comprise 1-2 % of primary nervous system tumours and 8-12 % of all posterior fossa lesions in the adult. They commonly occur in young adults with a peak incidence corresponding to the fourth decade of life.

Methods: Twenty-two patients with 24 posterior fossa hemangioblastomas were identified, accounting for 6.4 % of all posterior fossa tumours surgically treated from January 1980 to January 2006.

Results: There were 12 males and 10 females. The patients were aged from 18 to 58 years with an average age of 39.1. Tumors were located on cerebellar hemisphere (17), cerebellar vermis (4) and brain stem (1). Tumors were cyst in 16 cases, solid in 8 cases. Total tumor removal was achieved in 21 patients (95 %), and incomplete removal in 1 cases.

Conclusions: Hemangioblastomas are benign tumors of uncertain origin that are located predominantly in the posterior cranial fossa and the spinal cord. Although most hemangioblastomas are sporadic, they are associated with autosomally dominant von Hippel-Lindau (VHL) disease in approximately 25 % of cases. Most hemangioblastomas can be cured with surgical resection, and long-term recurrence rates seem to depend on the presence of VHL disease and multicentric lesions.