Supratentorial and infratentorial haemangioblastomas: literature review with lllustrative cases

ABSTRACT

The central nervous system haemangioblastomas are a unique group of life threatening, histologically benign tumours occurring solely within the neuroaxis. They are characterised by a familial incidence, a striking diversity of associated lesions in the organs and a gratifying response to microsurgical treatment. They are uncommon intracranial neoplasms constituting approximately 1 - 2% of intracranial masses and 7% of all infratentorial tumours. They may be associated with Von Hippel Lindau Complex [VHLC]. Among central nervous system haemangioblastomas, solitary or multiple, supratentorial haemangioblastomas are rare. They account for 2 - 8% of all haemangioblastomas. They are commonly intraparenchymatous, infrequently intraventricular and rarely in meningeal location. Interesting clinical, neuroradiological [computed tomography [CT] and magnetic resonance imaging [MRI]] findings are described in this review of literature with illustrative cases