Von Hippel-Lindau syndrome
Von Hippel-Lindau syndrome
Von Hippel-Lindau syndrome (VHL) is a hereditary condition associated with tumors arising in multiple organs. Tumors in VHL include hemangioblastomas, which are blood vessel tumors of the brain, spinal cord, and eye. The eye tumors are also called retinal angiomas. People with VHL also have an increased risk of developing clear cell renal cell carcinoma, which is a specific type of kidney cancer; pheochromocytoma, which is a tumor of the adrenal gland; and a type of pancreatic tumor known as pancreatic neuroendocrine tumor. Other features of VHL include: kidney cysts, which are closed sacs usually filled with fluid; pancreatic cysts, epididymal cystadenomas, which are tumors near a man’s testicles; and endolymphatic sac tumors, which are tumors of the ear that may cause hearing loss.
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