Von Hippel-Lindau syndrome

Angiomatosis retinae; Cerebelloretinal Angiomatosis, Familial; Hippel-Lindau Disease; VHL syndrome

... Von Hippel-Lindau syndrome is an inherited disorder characterized by tumors and fluid-filled sacs called cysts that can develop in many different parts of the body. Tumors may be either benign (noncancerous) or malignant (cancerous) and occur from early childhood to late adulthood.

Tumors called hemangioblastomas or angiomas can develop in the brain and spinal cord, the kidneys, and the back of the eye (retina). These tumors are made up of newly formed blood vessels and are typically noncancerous. Other types of tumors, which may be cancerous, often occur in the kidneys, the adrenal glands (hormone-producing glands located above each kidney), the pancreas, certain reproductive organs, and the inner ear.

Von Hippel-Lindau syndrome is sometimes divided into two types. Type 1 von Hippel-Lindau syndrome is associated with a high frequency of pheochromocytoma. This type of tumor, which is usually noncancerous, develops in the adrenal gland and can cause dangerously high blood pressure. Type 2 von Hippel-Lindau syndrome tends to involve fewer pheochromocytomas, but is associated with an increased risk of developing kidney cancer. ...

Von Hippel-Lindau disease (VHL) is a rare inherited genetic condition invol ...