Supratentorial intraventricular hemangioblastomas.

A 33-year-old male presented with a headache. He had a history of a previous surgical procedure for excisions of cervical spine hemangioblastomas 13 years prior He had a family history of von-Hippel Lindau (VHL) disease, and a VHL mutation was identified. Brain magnetic resonance imaging showed enhanced mass lesions in both the third and right lateral ventricles as well as in the cerebellum. The lesion in the right lateral ventricle also had a cystic component. Two-staged resections of the tumors in the third and right lateral ventricles and ventriculo-peritoneal shunt were performed. A histopathological specimen was compatible with a hemangioblastoma. Supratentorial intraventricular hemangioblastomas are extremely rare. We reviewed the literature and discussed the features.