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

Introduction: Hemangioblastomas (HGB) are rare, benign WHO grade 1 vascular tumor. Limited data are available regarding factors affecting outcomes following Stereotactic Radiosurgery (SRS), either in Von Hippel-Lindau (VHL) associated HGB or sporadic cases

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 having 433 and 137 tumors, respectively. The median age at SRS for VHL and sporadic patients was 35 and 51 respectively. Median follow up after initial SRS was 52 months and 44 months in VHL and sporadic, respectively

Results: At last, follow up, tumor control was achieved in 85% and 76% of VHL and sporadic tumors, respectively. Cystic tumors progressed more after SRS in sporadic cases (13 (9.6%)). Radiation induced changes were identified in 13 (3.5%), 5 (3.8%) of VHL and sporadic cases, respectively. Overall, 3-year and 5-year cumulative incidences of all tumor progression , VHL, Sporadic were 13%/22%, 14%/25% and 13%/17%, respectively. 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 to sporadic group. Multiple factors related to distant tumor control, local control, and overall survival were identified to be statistically significant.

Conclusion : SRS offers an effective treatment option for intracranial hemangioblastoma, whether VHL-associated or sporadic, with a favorable risk profile. Hemangioblastomas without a cystic component were more likely to be controlled after SRS