Surgical Treatment of Posterior Fossa Hemangioblastomas

Abstract

Background: Hemangioblastomas (HBL) are uncommon tumors of the central nervous system (CNS), corresponding to 1 to 2.5% of all intracranial tumors. They can present sporadically or in patients with von Hippel-Lindau (VHL) disease and are most often located in the cerebellum, brainstem, and spinal cord. VHL disease is a multiple neoplasia syndrome inherited in an autosomal dominant fashion and caused by a suppressor gene deletion. We present our experience in the management of patients with posterior fossa HBL.

Methods: Sixty-one consecutive patients with posterior fossa HBL were included in this study. Hospital charts, radiological images, and operative records were reviewed. Modified Rankin scores were used to evaluate the clinical course.

Results: Sixty-one patients (61% females, mean age: 51 years old) diagnosed with posterior fossa HBL, were operated with a mean follow-up of 72 months. Most frequent location corresponded to cerebellar hemisphere lesions (74%), followed by vermis and brainstem. Nine patients (17%) were diagnosed with VHL, consulting at a younger age, with a trend toward multiple lesions and the need for more than one surgical procedure. The most frequent clinical presentation corresponded to cerebellar syndrome (75%) followed by increased ICP symptoms. Hydrocephalus was detected in 35% of patients at the time of consultation. Gross-total resection was achieved in 93% of the cases. Only three patients underwent preoperative selective embolization. Mean hospital stay was 5 days. Complications observed included surgical site infection/CSF fistula (9.8%), cranial nerve palsy (8.2%), hydrocephalus (4.9%), and hemorrhage (6.6%). At last follow-up, 96% of the patients showed good functional recovery.

Conclusions: HBL of the posterior fossa should be resected in symptomatic patient or when the tumor (or a tumor-associated cyst) shows signs of enlargement. Surgical removal should be in-bloc resection to minimize intraoperative bleeding. Patients with HBLs must be tested for gene mutations, and in confirmed cases, relatives should be offered genetic counseling.