What Is von Hippel–Lindau Disease?

Von Hippel–Lindau (VHL) disease is a rare inherited condition that increases the risk of developing tumors in different parts of the body, including the kidneys. It is caused by a mutation in the VHL gene, which normally helps regulate cell growth. When this gene is not working correctly, abnormal cell growth can occur, leading to tumors.

People with VHL disease are at a higher risk of developing clear cell renal cell carcinoma, the most common type of kidney cancer in adults. These kidney tumors can appear in both kidneys (bilateral) or as multiple tumors in one kidney, often at a younger age than kidney cancers seen in the general population.

In addition to kidney cancer, VHL disease can also lead to:

  • Hemangioblastomas (tumors in the brain and spinal cord)
  • Pheochromocytomas (tumors in the adrenal glands)
  • Pancreatic cysts or tumors
  • Retinal angiomas (tumors in the eyes)

Because of the wide range of possible tumor locations, people with VHL need regular monitoring using imaging tests such as MRI or CT scans. Early detection of tumors can help prevent complications and guide treatment decisions.

VHL is inherited in an autosomal dominant pattern, which means a person only needs one copy of the faulty gene to be affected. If a parent has VHL, there is a 50% chance of passing it on to their child.

There is no cure for VHL, but early diagnosis and ongoing surveillance can help manage the condition and improve outcomes. Genetic counseling and testing are often recommended for families affected by VHL to help identify who is at risk and ensure proper care.

Looking for more questions?

Visit our Kidney Cancer Questions page for all other topics explained in plain language.

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