P12.02BRAINSTEM HEMANGIOBLASTOMAS IN PATIENTS WITH VON HIPPEL-LINDAU DISEASE - SURGICAL CONSIDERATIONS

BACKGROUND: Von Hippel-Lindau disease (VHL) predisposes to the development of CNS hemangioblastomas. Brainstem hemangioblastomas present in 15 to 30% of VHL patients, particularly in the medulla. Hemangioblastomas at this location are characteristic and almost exclusive for VHL. There is scarce information about the most adequate management of these particular tumors. The objective of this work is to analyze the results of treatment at a VHL dedicated unit and following personal surgical criteria. MATERIAL AND METHODS: Since 2001 we have studied and treated 105 patients with VHL disease. Thirty three have presented brainstem hemangioblastomas. A microsurgical resection was performed of tumors when symptomatic and/or growing to a diameter of 1.5 cm. In all cases, neuronavigation and neurophysiology intraoperative monitorization (NIM) were used. All patients have a postoperative clinical and imaging follow up of at least 12 months (mean 47.37 m). Clinical evaluation has been monitored with McCormick and Karnofsky scales before surgery, at immediate postoperative, and 1, 6 and 12 months later. RESULTS: Twenty one surgical procedures were performed in sixteen patients, 9 males and 7 women, with mean age of 40.6 y (range 15-49 y), for resection of 26 brainstem hemangioblastomas. Mean tumor maximal diameter was 17.9 mm. Most frequent symptoms were sensory and motor disturbances, dizziness, and unsteadiness. Some cases presented very particular symptoms, as recurrent hiccup, persistent nausea, and dysphagia. Three patients were asymptomatic, and surgery was proposed because of rapid tumor growth up to more than 15 mm. All patients did well, improving their symptoms and maintaining or improving their functional level. CONCLUSIONS: The resection of brainstem hemangioblastomas in VHL patients can be followed by very good results, when indicated with specific criteria and performed in expert units. Neuronavigation and NIM are recommended tools for accurate and safe procedures. We recommend brainstem hemangioblastoma surgery just at the beginning of symptoms, and/or when tumor diameter grows equal to or exceeds 15 mm, in order to maximize the preservation of brainstem tissue and nearby vasculature shared by the tumor, providing the best functional recovery.