von Hippel-Lindau Disease
Definition
Von Hippel-Lindau disease (VHL) is a rare familial cancer syndrome. A person with VHL can develop both benign and malignant tumors and cysts in many different organs in the body. Tumors and cysts most commonly develop in the brain and spine, eyes, kidneys, adrenal glands, pancreas, and inner ear.
Description
VHL does not have a predictable set of symptoms. VHL affects approximately 1 in 35,000 people, and affects men and women equally. Some families may have different symptoms than other families. Even within a family, there may be people with very mild signs of VHL, and others with more severe medical problems. The age when symptoms develop can range from infancy to late adulthood, although most people with VHL will have some clinical symptoms by age 65. It is...
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