495 Delineating Growth Dynamics and Surgical Indicators in VHL-Associated and Sporadic Hemangioblastomas

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INTRODUCTION: Hemangioblastomas, especially those with von Hippel-Lindau (VHL) disease, exhibit variable clinical trajectories, ranging from prolonged stability to aggressive growth necessitating surgical intervention. METHODS: We retrospectively identified patients with intracranial hemangioblastomas resected between 2000 and 2022 at a single institution. Patient demographics, VHL status, and symptoms were obtained through chart review. Disease burden and tumor characteristics, including location, peritumoral edema, and cystic morphology, were collected across serial imaging. Tumor volume was determined using 3D contouring software on neuroimaging (Elements SmartBrush, Brainlab AG, Munich) of enhancing tissue only. RESULTS: We analyzed 260 hemangioblastomas (70 sporadic resected, 83 VHL-associated resected, 107 VHL-associated monitored) in 122 patients with 15 years of followup. Similar numbers of hemangioblastomas grew between resected and monitored cohorts (71.2% vs. 65.3% respectively, p = 0.407), Growth patterns were categories as exponential, linear, saltatory, other, or no growth. Analysis of 105 lesions (26 resected, 79 monitored) showed resected tumors primarily grew exponentially (73.1%) compared to linearly (11.5%). Monitored tumors typically remained stable (41.8%) or grew exponentially (34.2%). Tumors undergoing resection were more likely to be edematous (p < 0.001), cystic (p < 0.001), larger (p < 0.001), located in the vermis (p = 0.027), and grow exponentially (p = 0.004). Cervicomedullary lesions underwent surgery more often (33.3% resected, p = 0.046) compared with other locations. Across both VHL and sporadic cohorts, sex, race, and surgical history were not associated with surgical resection (p = 0.169, p = 0.720, p = 0.154, respectively). CONCLUSIONS: This study highlights the growth behaviors of sporadic and VHL-associated hemangioblastoma. The decision to pursue surgery is influenced by tumor characteristics such as size, edema, and growth.

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