Predictors of Progression in a Series of 81 Adult Patients Surgically Managed for an Intracranial Hemangioblastoma: Implications for the Postoperative Follow-Up

Simple Summary Local or distant progression is possible after the resection of an intracranial hemangioblastoma. Few studies have focused on predictors of hemangioblastoma progression, especially for intracranial locations. Therefore, the aim of the present study was to precisely identify the predictors of local and distant progression in a series of 81 patients managed for an intracranial hemangioblastoma in order to ultimately tailor the follow-up to each patient profile. As about a quarter of the patients with sporadic hemangioblastoma would have a local progression, with a progression-free survival of 56 months following surgery, it would be advisable to plan a regular surgical and radiological screening for at least 10 years postoperatively. The local recurrence can be particularly quick in the case of partial resection, which justifies closer radiological monitoring. In patients with von Hippel–Lindau disease, annual monitoring has to be planned indefinitely given the risk of local and distant progression. Abstract The aim was to identify predictors of progression in a series of patients managed for an intracranial hemangioblastoma, in order to guide the postoperative follow-up modalities. The characteristics of 81 patients managed for an intracranial hemangioblastoma between January 2000 and October 2022 were retrospectively analyzed. The mean age at diagnosis was of 48 ± 16 years. Eleven (14%) patients had von Hippel–Lindau disease. The most frequent tumor location was the cerebellar hemispheres (n = 51, 65%) and 11 (14%) patients had multicentric hemangioblastomas. A gross total resection was achieved in 75 (93%) patients. Eighteen (22%) patients had a local progression, with a median progression-free survival of 56 months 95% CI [1;240]. Eleven (14%) patients had a distant progression (new hemangioblastoma and/or growth of an already known hemangioblastoma). Local progression was more frequent in younger patients (39 ± 14 years vs. 51 ± 16 years; p = 0.005), and those with von Hippel–Lindau disease (n = 8, 44% vs. n = 3, 5%, p < 0.0001), multiple cerebral locations (n = 3, 17% vs. n = 2, 3%, p = 0.02), and partial tumoral resection (n = 4, 18% vs. n = 1, 2%, p = 0.0006). Therefore, it is advisable to propose a postoperative follow-up for at least 10 years, and longer if at least one predictor of progression is present.