Man with posterior fossa tumors 15 years apart

A 48-year-old man with a history of hypertension, peripheral vascular disease and a 50-pack-year history of smoking presented with new onset vertigo, tinnitus, diplopia and ataxia in January 1978. CT scan showed a radiolucent defect in the left cerebellar hemisphere with a possible mural nodule. In ensuing months, he experienced worsening symptoms with a corresponding increase in lesion size on re-imaging. Months later, a left posterior fossa craniotomy was performed and revealed a single cystic lesion containing copious amounts of straw-colored fluid and a single mural nodule in the inferior portion of the cyst. Following resection, he was followed clinically until 1985 at which time follow-up was discontinued. He did well until January 1993 when he presented with progressively worsening episodic headaches and retro-orbital pressure. Subsequent MRIs of the brain and spine (February 1993, March 1993) showed multiple lesions along the neuraxis involving the superficial brain parenchyma, leptomeninges, and dura. Despite therapy, his condition progressively declined until he succumbed. A brain-only autopsy revealed numerous small tumor nodules involving the base of the brain over both frontal and temporal lobes, midbrain, pons, right optic nerve, pituitary fossa, and the base of the skull. Pathologic evaluation revealed metastatic hemangioblastoma. Metastatic hemangioblastoma is a rare entity, with only a few reported cases in the literature to date.