gms | German Medical Science

Objective: Hemangioblastomas (HBL) are benign neoplasms contributing to 1-2.5% of all intracranial tumors and to 7-12% of posterior fossa lesions in adults. HBL evolve either hereditarily in association with von Hippel-Lindau disease (vHL) or, more prevalently, as solitary sporadic tumors. Few authors reported data concerning clinical presentation and neurological outcome of hereditary and sporadic HBL. We extracted patients with HBL of the posterior cranial fossa that have been treated at our center during the past ten years.

Method: We retrospectively analyzed 24 consecutive patients (11 male, 13 female; mean age 51.3 years) with HBL involving the posterior cranial fossa between 2001 and 2012. The clinical, radiologic, surgical, and pathologic records were reviewed to evaluate radiologic findings, clinical symptoms, duration of symptoms, manifestations of vHL, surgical approach, adhesive structures, duration of surgical procedure, extent of resection (total, or near total), complications, recurrence rates, and final outcomes (according to modified Rankin scale; mRS). The mean follow-up was 21 months.

Results: 21 HBL were located in the cerebellum, and 2 were harbored in the craniocervical junction. Mean tumor size was 368 mm. The major symptoms were headache, vertigo/ataxia, and nausea. Mean time to diagnosis was14 weeks. 1 patient had a positive family history of HBL, 3 patients fulfilled the criteria for VHL disease, 12 had sporadic HBL. 3 patients required ventricular shunting preoperatively and 1 patient intraoperatively. Strong intraoperative bleeding was reported in 5 cases and 2 blood transfusions were necessary. Cortical adherences were reported in 3 cases. The extent of resection (EOR) was total in 21, and near total in 3 patients. Within 24 hours postoperatively, revision was necessary due to relevant bleeding in 4 patients. 1 patient died within 14 days. 1 patient required permanent shunting. At discharge, 3 patients presented with persistent ataxia, 1 patient with new diplopia and 1 patient with a new hemiparesis. 75% (n=18, mRS 0-1) had no or improved symptoms. 3 recurrences were detected during follow-up.

Conclusions: In contrast to other benign entities of the posterior fossa, the time to diagnosis was significantly shorter in HBL. This finding may reflect a rather aggressive biological behavior of these excessively vascularized tumors. However, a relatively high rate of complete resection was achieved in our series with a morbidity and mortality that were within the reported range.