Von hippel-lindau disease (vhl)
Von Hippel-Lindau disease (VHL) is a rare, genetic multi-system disorder in which non-cancerous tumors grow in certain parts of the body.
Pathophysiology[edit | edit source]
- Slow-growing hemgioblastomas which are 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 cancers[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. Death is usually caused by complications of brain tumors or kidney cancer.
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 by putting pressure on the brain or spinal cord.
- This pressure can block the flow of cerebrospinal fluid in the nervous system, impair vision, or create deafness.
- Treatment of most cases of VHL usually involves surgery to remove the tumors before they become harmful. Certain tumors can be treated with focused high-dose irradiation.
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