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Von Hippel-Lindau Disease
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Genetics Birth Defects

Von Hippel-Lindau Disease

Von Hippel-Lindau disease (VHL) is a rare genetic condition characterized by the growth of tumors and cysts throughout the body.

What is Von Hippel-Lindau disease (VHL)?

Von Hippel-Lindau disease (VHL) is a rare disease that causes tumors and cysts to grow in your body. They can grow in your brain and spinal cord, kidneys, pancreas, adrenal glands, and reproductive tract. The tumors are usually benign (non-cancerous). But some tumors, such as those in the kidney and pancreas, can become cancerous.

What causes Von Hippel-Lindau disease (VHL)?

VHL is a genetic disease. It is inherited, which means that it is passed down from parent to child.

What are the symptoms of Von Hippel-Lindau disease (VHL)?

Symptoms of VHL depend on the size and location of the tumors. They may include:

  • Headaches
  • Problems with balance and walking
  • Dizziness
  • Weakness of the limbs
  • Vision problems
  • High blood pressure

How is Von Hippel-Lindau disease (VHL) diagnosed?

Detecting and treating VHL early is important. Your health care provider may suspect that you have VHL if you have certain patterns of cysts and tumors. There is a genetic test for VHL. If you have it, you will need other tests, including imaging tests, to look for tumors and cysts.

What are the treatments for Von Hippel-Lindau disease (VHL)?

Treatment can vary, depending on the location and size of the tumors and cysts. It usually involves surgery. Certain tumors may be treated with radiation therapy. The goal is to treat growths while they are small and before they do permanent damage. You will need to have careful monitoring by a doctor and/or medical team familiar with the disorder.

Frequently Asked Questions

Individuals with a confirmed VHL mutation require lifelong surveillance, typically starting in childhood. This includes annual MRI scans of the brain and spine to detect hemangioblastomas, and annual abdominal CT or MRI scans to monitor the kidneys, pancreas, and adrenal glands. Blood pressure checks are also performed regularly, as high blood pressure can be an early sign of a renal cell carcinoma.
There is no cure for VHL because it is a genetic condition affecting the DNA. However, the prognosis is generally good with early detection and treatment. The goal of management is to remove or treat tumors before they grow large enough to cause permanent organ damage or become malignant. With consistent monitoring and timely intervention, many individuals with VHL live normal life spans.
VHL can cause hemangioblastomas in the retina (the light-sensitive layer at the back of the eye). These growths can lead to vision loss if they bleed or cause fluid to accumulate behind the retina. Symptoms may include floaters, flashes of light, or a sudden decrease in vision. Regular eye exams with dilation are crucial for early detection, and treatment often involves laser therapy or anti-VEGF injections to preserve sight.
While there is no specific diet that prevents tumor growth in VHL, maintaining a healthy weight and managing blood pressure are critical. A low-sodium diet can help control hypertension, which is a common complication. Avoiding smoking is essential, as it increases the risk of cardiovascular disease and may complicate surgical recovery. Regular physical activity is encouraged to support overall health, but patients should consult their care team before starting new exercise routines.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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