• Highly vascularized tumor composed of stromal cells and capillaries usually found in the cerebellum, brain stem, or spinal cord

Clinical Features


  • 1% to 2% of all intracranial tumors

  • Most common in adults in third to fifth decades; can affect infants and children especially in association with von Hippel-Lindau (VHL) disease

  • No gender predominance

  • Majority are sporadic

  • 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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