Leptomeningeal hemangioblastoma: illustrative case

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BACKGROUND: Hemangioblastoma is a slow-growing vascular tumor commonly found in the posterior fossa. It is associated with von Hippel-Lindau disease, yet most cases arise sporadically. Resection and belzutifan are highly effective in the treatment of hemangioblastoma. Rarely, leptomeningeal dissemination may occur months to years after resection. OBSERVATIONS: A 56-year-old female with cerebellar hemangioblastoma resected in 2016 presented 8 years later with left hemiparesis. Updated neuroimaging revealed new leptomeningeal disease, confirmed via tissue from a right cerebellopontine angle mass. The authors conducted a review of 33 patients diagnosed with leptomeningeal disease; two-thirds were male with a median age of 48 years. Thirty-two patients presented initially with a solitary mass, and 96% of these underwent resection. The mean time from initial lesion resection to dissemination was 94 months. LESSONS: After resection, hemangioblastoma recurrence with leptomeningeal spread is a rare but dangerous possible complication. Management is complex, yet a combination of pan-CNS radiation therapy, targeted resection, and belzutifan (a small-molecule inhibitor that selectively targets and blocks the function of HIF-2α) may represent an effective treatment combination. https://thejns.org/doi/10.3171/CASE25204.

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