Surgical resection of sporadic and hereditary hemangioblastoma: Our 10-year experience and a literature review

Abstract

Background: Hemangioblastomas (HBLs) are benign neoplasms that contribute to 1-2.5% of intracranial tumors and 7-12% of posterior fossa lesions in adult patients. HBLs either evolve hereditarily in association with von Hippel-Lindau disease (vHL) or, more prevalently, as solitary sporadic tumors. Only few authors have reported on the clinical presentation and the neurological outcome of HBL.

Methods: We retrospectively analyzed the clinical, radiological, surgical, and histopathologic records of 24 consecutive patients (11 men, 13 women; mean age 51.3 years) with HBL of the posterior cranial fossa, who had been treated at our center between 2001 and 2012. We reviewed the current literature, and discussed our findings in the context of previous publications on HBL. The study protocol was approved by the local ethics committee (14-101-0070).

Results: Mean time to diagnosis was 14 weeks. The extent of resection (EOR) was total in 20 and near total in 4 patients. Four patients required revision within 24 h because of relevant postoperative bleeding. One patient died within 14 days. One patient required permanent shunting. At discharge, 75% of patients [n = 18, modified Rankin scale (mRS) 0-1] showed no or at least resolved symptoms. Mean follow-up was 21 months. Two recurrences were detected during follow-up.

Conclusions: In comparison to other benign entities of the posterior fossa, time to diagnosis was significantly shorter for HBL. This finding indicates the rather aggressive biological behavior of these excessively vascularized tumors. In our series, however, the rate of complete resection was high, and morbidity and mortality rates were within the reported range.

Keywords: CNS hemangioblastoma; neurological outcome; posterior cranial fossa; von Hippel–Lindau disease.

Figure 1

Age distribution by decades of patients with hemangioblastoma in the posterior cranial fossa

Figure 2

Mean time to diagnosis in the entire cohort (n = 24, ranging from 1 day to 57 weeks) in the subgroup with sporadic hemangioblastoma (HBL) (n = 21) and in the subgroup with hereditary HBL [von Hippel-Lindau disease (vHL), n = 3, ranging from 4 to 12 weeks]. Analyses showed a considerably shorter mean time from the onset of symptoms to diagnosis in patients with vHL disease-associated HBL

Figure 3

Characteristics of available preoperative MRI of 12 out of 24 patients with hemangioblastoma. MN: Mural nodule

Figure 4

(a) T1-weighted, axial and (b) T2-weighted, axial MRI of a cystic hemangioblastoma localized in the left cerebellar hemisphere, showing the characteristic mural nodule with strong gadolinium enhancement. (c) Postoperative MRI (T1-weighted, axial), confirming total tumor removal and resection of the cystic component after retrosigmoid suboccipital craniotomy. The space-occupying effect and the brain stem compression are alleviated