Solitary hemangioblastoma of the fourth ventricle in an adult male

Abstract

Abstract Hemangioblastoma is a rare, benign and slow-growing tumor of uncertain histogenesis. It constitutes roughly 1% to 2% of all intracranial tumors and most commonly arises in the posterior cranial fossa: more than 80% of hemangioblastomas are located in the cerebellar hemispheres, the remaining cases lie in the vermis, brainstem and spinal cord. In about 20% of the cases hemangioblastomas are associated with the hereditary disorder known as Von Hippel-Lindau disease (VHL). Usually, hemangioblastomas presents with symptoms of increased intracranial pressure and focal neurological symptoms, which depend on the tumor location. Solitary intraventricular hemangioblastomas are exceptionally rare, only two documented cases of fourth ventricular tumors have been reported so far, one with an association of phaeochromocytoma and renal medullary fibroma and the other as solitary fourth ventricular tumor, here we describe a case with solitary fourth ventricular hemangioblastoma which was completely excised. A thorough generalised survey with both clinical and radiological methods revealed that this case was a sporadic isolated case of hemangioblastoma in the fourth ventricle.