Hemangioblastoma: Sporadic Cases and Von Hippel-Lindau's Disease: About 4 Cases and Literature Review

Authors: Khay H*, Raouzi N, Khoulali M, Oulali N, Moufid F

DOI: 10.23880/ijsst-16000147

Volume 4, Issue 2

Abstract

Hemangioblastomas (HGB) are rare tumors of the central nervous system (CNS) that originate from the vascular system. They represent 2 to 3% of brain tumors. The infratentorial (cerebellum, elongated marrow) or spinal (spinal marrow) are the locations of preferences (99%). These tumors account for 10% of all posterior cranial fossa brain tumors and are the most common primary posterior cranial fossa brain tumors in adults. Two-thirds of hemangioblastomas occur sporadically, onethird of cases in the context of familial neoplasia syndrome, von Hippel-Lindau disease (VHL). More than 80% of VHL patients will develop CNS hemangioblastoma during their lifetime and most of them (95%) will develop multiple hemangioblastomas. We report four cases of cerebelous hemangioblastoma treated in our neurosurgery department: two child brothers with VHL disease, from a mother treated for cerebelous hemangioblastoma, and two adults with sporadic hemangioblastoma. The 2 cases of hemangioblastomas in the context of VHL disease were, respectively, 13 years (Girl) and 22 years old (boy), from a mother with VHL disease and in both cases the first complaint was the symptoms of intra cranial hypertension syndrome associated with a cerebellar syndrome. Radiological exploration shows a cerebellar location for both cases associated with cervical spinal lesion in the case of the girl. Two sporadic male cerebellar hemangioblastomas cases aged 26 and 48, respectively. The 4 cases benefited from surgical exeresis with good evolution.

Keywords: Hemangioblastoma; Sporadic; Von Hippel-Lindau Disease; Cerebellum; Surgery; Treatment

View PDF