A Robust Allegation of Von Hippel-Lindau (VHL) Associated Hemangioblastoma in the Central Nervous System (CNS): A Case Report

Abstract

Haemangioblastoma, a benign vascular tumor derived from the capillary of endothelial cells, is an inherited disorder due to an autosomal dominant trait. The prevalence rates accounted for 1% to 2.5% of all intracranial tumors and 2% to 3% of all intramedullary spinal cord tumors. We report the infrequent case of a robust allegation of von Hippel-Lindau (VHL) associated haemangioblastoma in the central nervous system of a man. A 20 years old male presented to the hospital with the chief complaint of progressive chronic cephalgia three months prior to hospitalization. He complained also of blurred vision on neurologic examination; there is papilla edema with suspicion of retinal edema. Radiological examination result suggested a cerebellar haemangioblastoma dextra with noncommunicating hydrocephalus. After the first operation (installation of VP shunting) and subsequent operation (excision of the tumor), the pathological anatomy results revealed a cavernous angioma.