Leptomeningeal hemangioblastoma: illustrative case
Year: 2025
Type: article
Abstract: 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.
DOI: 10.3171/case25204
Authors: Joshua M. Venegas, Noah L. A. Nawabi, Aaron Miller, Tiffany Baker, Scott Lindhorst, Alicia Zukas, Charlotte Rivers, William Vandergrift, Ben A. Strickland
Institution: Medical University of South Carolina
Language: English
Topic: Ear and Head Tumors
Subfield: Oncology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
OpenAlex ID: W4411333699