Angiomatosis
Other names[edit | edit source]
Von Hippel-Lindau disease (VHL)
Pathophysiology[edit | edit source]
It is a rare, genetic multi-system disorder in which non-cancerous tumors grow in certain parts of the body.
Types of tumors[edit | edit source]
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[edit | edit source]
- 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[edit | edit source]
Individuals with VHL are also at a higher risk than normal for certain types of cancer, especially kidney cancer.
Prognosis[edit | edit source]
- 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[edit | edit source]
- 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[edit | edit source]
Hippel-Lindau Disease (VHL)-Information-Page Von Hippel-Lindau Disease (VHL) at NINDS
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