Outcomes After Stereotactic Radiosurgery for Intracranial Hemangioblastoma in Von Hippel-Lindau Disease and Sporadic Cases: An International Multicenter Study

BACKGROUND AND OBJECTIVES: Hemangioblastomas (HGBs) are rare, benign, World Health Organization grade 1 vascular tumors, which are most commonly located in the cerebellum, and may occur sporadically or in association with von Hippel–Lindau (VHL) disease, a hereditary VHL-mutated tumor syndrome. Limited data are available regarding factors affecting outcomes after stereotactic radiosurgery (SRS). We aim to provide a contemporary evaluation of SRS for HGBs through an international, multicenter study. In this study, we assess local tumor control and SRS-related complications in patients with intracranial HGBs. METHODS: A retrospective analysis from 17 centers was performed. Data on patient characteristics, SRS parameters, and outcomes were collected. The study included 104 patients with VHL and 89 sporadic cases, with 433 and 137 tumors, respectively. The median follow-up after the initial SRS was 52 months for patients with VHL and 44 months for sporadic cases. RESULTS: At the last follow-up, tumor control was achieved in 85% of VHL tumors and 76% of sporadic tumors. Radiation-induced changes were identified in 13 (3.5%) VHL cases and 5 (3.8%) sporadic cases. The overall 3-year and 5-year cumulative incidences of tumor progression were 13% and 22% for all tumors, 14% and 25% for VHL, and 13% and 17% for sporadic cases. Tumor progression was more common in cystic than in solid tumors in the sporadic group. Overall survival probability and progression-free survival were better in VHL cases compared with the sporadic group. Older age at SRS, male sex, and multiple tumors were associated with reduced local tumor control in all tumors and in the VHL group, whereas a margin dose >15 Gy was associated with improved local tumor control in both groups. CONCLUSION: SRS offers an effective treatment of intracranial HGBs, whether VHL-associated or sporadic, with a favorable risk profile. HGBs without a cystic component were more likely to be controlled after SRS in the sporadic group.