An autosomal dominant disorder caused by mutations in a tumor suppressor gene. This syndrome is characterized by abnormal growth of small blood vessels leading to a host of neoplasms. They include hemangioblastoma in the retina; cerebellum; and spinal cord; pheochromocytoma; pancreatic tumors; and renal cell carcinoma (see carcinoma, renal cell). Common clinical signs include hypertension and neurological dysfunctions.

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