Clinical Features and Surgical Outcomes in Patients with Cerebellopontine Angle Hemangioblastomas: Retrospective Series of 23 Cases.

Abstract

Hemangioblastomas in the cerebellopontine angle (CPA) are uncommon and have rarely been reported. They may be easily misdiagnosed because of the atypical location and clinical and imaging features. The present study aimed to characterize clinical and radiologic features, treatment strategies, and outcomes in these rare lesions and to investigate various factors that may affect postoperative outcomes. The medical records of patients with CPA hemangioblastomas who underwent surgery from 2003-2016 at the West China Hospital were reviewed retrospectively and statistically analyzed. Twenty-three patients (14 males and 9 females) presented with CPA hemangioblastomas. Eight patients (34.8%) had von Hippel-Lindau (VHL) syndrome. Gross total resection was achieved in 22 patients (95.6%). The mean follow-up was 45.1 ± 36.2 months (range 3-144 months). After surgery, the symptoms improved in 18 cases (78.3%), remained unchanged in 3 cases (13%), and were aggravated in 2 cases (8.7%). Four patients showed local recurrence during follow-up (17.4%). Patients with cystic hemangioblastomas had a better neurologic improvement (P = 0.041) compared with patients with solid tumors. Furthermore, patients with maximal diameter of tumors >3 cm (P = 0.035) or solid tumors (P = 0.018) showed a higher incidence of postoperative complications. The local recurrence was correlated with VHL disease (P = 0.027). Although hemangioblastomas of the CPA are challenging lesions to treat surgically, they can be removed safely when these lesions are appropriately diagnosed and treated. Patients with VHL disease are more likely to have a local recurrence. A regular follow-up is recommended to detect the local and distant recurrence, even if the clinical course is benign and the tumor is totally resected.