von Hippel–Lindau disease (VHL), is a raregenetic disorder with multisystem involvement.It is characterized by visceral cysts and benign tumorswith potential for subsequent malignant transformation. It is a type of phakomatosis that results from a mutation in the von Hippel–Lindau tumor suppressor gene on chromosome , Signs and symptoms associated with VHL disease include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems, and high blood pressure. Conditions associated with VHL disease include angiomatosis, hemangioblastomas, pheochromocytoma, renal cell carcinoma, pancreatic cysts (pancreatic serous cystadenoma), endolymphatic sac tumor, and bilateral papillary cystadenomas of the epididymis (men) or broad ligament of the uterus (women).
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