Hemangioblastoma Cysts: Diagnosis Using Fluorescence with 5-Aminolevulinic Acid

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Hemangioblastoma is a benign tumor which often emerges sporadically in adult cerebellum or is associated with von Hippel-Lindau disease. Though it can be surgically removed, in rare occasions it can reappear, necessitating a second treatment to remove the tumor. Recurrences of hemangioblastoma are located either in an area removed from the initial tumor site or locally due to an incomplete excision of the tumor. The latter recurrence can be controlled by confirming that none of the tumor remains at the time of surgery. Intraoperative fluorescence diagnosis using 5-aminolevulinic acid (5-ALA) utilizes of protoporphyrin IX (PpIX) accumulation in tumor cells, resulting in PpIX accumulation in residual tumor cells which can be seen reacting under ultraviolet light. This is a simple, non-invasive technique to detect the presence of any residual tumor while still in the operating room. Although there are no tumor cells in cysts of hemangioblastoma, there are cases where a recurrence is thought originate from a residual cyst. Because rupturing these thin-walled cysts can cause damage to the surrounding healthy cerebellum, routine extirpation of hemangioblastoma cysts is not recommended unless the cysts have been invaded by tumor cells, in which case extirpation is necessary to prevent recurrence. Intraoperative fluorescence diagnosis using 5-ALA is a useful way to determine the presence of tumor cells in cyst walls associated with hemangioblastoma.