Hemangioblastomas are the most common primary intra-axial tumors of the adult posterior fossa and the tumors most often associated with von Hippel-Lindau disease. Resection of cerebellar hemangioblastomas involves tumor excision and drainage of associated cysts. The cyst wall is considered devoid of tumor cells and is not excised. We present an unusual variant of a hemangioblastoma that showed pathological evidence of a tumor within the cyst wall that correlated with radiographic cyst wall enhancement in a patient with a recurrent hemangioblastoma.CLINICAL PRESENTATION
A 38-year-old woman with von Hippel-Lindau disease presented with a recurrent cerebellar hemangioblastoma despite two previous operations during which the mural nodule was removed but the cyst wall was not. Magnetic resonance imaging showed a cystic lesion with an enhancing mural nodule with atypical nodular enhancement throughout the cyst wall.INTERVENTION
Because the patient had a history of multiple recurrences, gross total resection of the mural nodule and cyst wall was performed. At surgery, neovascularization and neoplasia within the cyst wall were visualized. Histopathological examination showed a capillary hemangioblastoma with tumor tissue inside the cyst wall. No clinical or radiographic evidence of recurrence was observed during 2 years of follow-up monitoring.CONCLUSION
This unusual case of a hemangioblastoma with cyst wall enhancement demonstrates a correlation between enhancement on magnetic resonance imaging and presence of neoplasia within the cyst wall, as well as the importance of considering complete resection of these areas, including the cyst wall, for the prevention of recurrence.