| umls-concept:C0206734 | skos:definition | MSH: A benign tumor of the nervous system that may occur sporadically or in association with VON HIPPEL-LINDAU DISEASE. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with INTRACRANIAL HYPERTENSION, and ataxia. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2071-2),NCI: A WHO grade I tumor of uncertain histogenesis which is composed of stromal cells and abundant capillaries. Approximately 25% of hemangioblastomas are associated with Von Hippel-Lindau disease, a disease inherited through an autosomal dominant trait and characterized by the develo | lld:umls |
