Supratentorial hemangioblastoma without von Hippel-Lindau syndrome in an adult

Hemangioblastomas (HBLs) are highly vascular and cystic benign neoplasms. They form very small part of intracranial tumours and are often localized in the posterior fossa. Although most of them are sporadic, a significant group is accompanied with von Hippel-Lindau (VHL) syndrome. This case report presents a 57-year-old woman treated with total resection using micro-surgical technique and was diagnosed as HBL based on histopathologic findings. Contrast-enhanced cranial MRI of the patient with the complaints of spasms in the right side of body showed a right paracentral mass that caused midline shift. In literature, the previously reported cases of supratentorial HBL unaccompanied with VHL syndrome were searched in PUBMED, compiled and presented. It should be borne in mind that rare HBLs manifesting with various neurological symptoms may occur in the supratentorial region, and may not accompany with VHL syndrome.