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Hemangioblastoma
Definition
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Highly vascularized tumor composed of stromal cells and capillaries usually found in the cerebellum, brain stem, or spinal cord
Clinical Features
Epidemiology
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1% to 2% of all intracranial tumors
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Most common in adults in third to fifth decades; can affect infants and children especially in association with von Hippel-Lindau (VHL) disease
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No gender predominance
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Majority are sporadic
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25% are associated with von Hippel-Lindau disease (autosomal-dominant inherited defect in VHL gene on 3p25-p26; multiple hemangioblastomas, renal cell carcinoma, pheochromocytoma, cysts of pancreas and liver, endolymphatic sac tumor)
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