Angiomatosis

Other names

Von Hippel-Lindau disease (VHL)

Pathophysiology

It is a rare, genetic multi-system disorder in which non-cancerous tumors grow in certain parts of the body.

Types of tumors

Slow-growing hemgioblastomas -- benign tumors with many blood vessels -- may develop in the brain, spinal cord, the retinas of the eyes, and near the inner ear.

• Cysts (fluid-filled sacs) may develop around the hemangioblastomas.
• Other types of tumors develop in the adrenal glands, the kidneys, or the pancreas.

Symptoms

• Symptoms of VHL vary among individuals and depend on the size and location of the tumors.
• Symptoms may include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems, deafness in one ear, and high blood pressure.

Risk of cancer

Individuals with VHL are also at a higher risk than normal for certain types of cancer, especially kidney cancer.

Prognosis

• The prognosis for individuals with VHL depends on then number, location, and complications of the tumors.
• Untreated, VHL may result in blindness and/or permanent brain damage. With early detection and treatment the prognosis is significantly improved.

Treatment

• Treatment for VHL varies according to the location and size of the tumor.
• In general, the objective of treatment is to treat the tumors before they grow to a size large enough to cause permanent problems

Sources

Hippel-Lindau Disease (VHL)-Information-Page Von Hippel-Lindau Disease (VHL) at NINDS