Objective: Purpose of this paper is to describe the variation of surgical plan and technique required in a rare subset of hemangioblastomas compared to the accepted general strategy. The established rule in hemangioblastoma surgery is to avoid cyst wall removal, as it is not neoplastic, and it will not recur if mural nodule is completely removed. However, the wall of the associated cyst is occasionally enhanced by gadolinium on preoperative MRI. Methods: We present the case of a patient with a hemangioblastoma that was progressively compressed by a growing cyst, with the final appearance of a contrast-enhanced cyst wall. We collected similar cases reported in the literature. Results: Our study points out the need for a tailored pre-operative strategy, the usefulness of intraoperative fluorescent dyes, and the crucial role of frozen section histopathological analysis to confirm diagnosis and optimize treatment. Conclusions: When a hemangioblastoma is associated with a contrast-enhanced cyst, care must be taken to intraoperatively confirm the presence of neoplastic cells, and eventually remove the neoplastic cyst wall to reduce the risk of recurrence.
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