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Von Hippel-Lindau Syndrome

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What is von Hippel-Lindau syndrome?

Von Hippel-Lindau syndrome (VHL) is a hereditary condition associated with hemangioblastomas (blood vessel tumors) of the brain, spinal cord, and eye. The eye tumors are also called retinal angiomas. People with VHL also have an increased risk of developing clear cell renal cell carcinoma (a specific type of kidney cancer) and pheochromocytoma (a tumor of the adrenal gland). Kidney cysts (a closed sac usually filled with fluid), pancreatic cysts, epididymal cystadenomas (tumors near a man’s testicles), and endolymphatic sac tumors (tumors of the ear, which may cause hearing loss) are also features of VHL.

What causes VHL?

VHL is a genetic condition. This means that the cancer risk and other features of VHL can be passed from generation to generation in a family. The gene associated with VHL is also called VHL. A mutation (alteration) in the VHL gene gives a person an increased risk of developing kidney cancer and other symptoms of VHL. Nearly everyone who has VHL syndrome has an identifiable VHL mutation.

How is VHL inherited?

Normally, every cell has two copies of each gene: one inherited from the mother and one inherited from the father. VHL follows an autosomal dominant inheritance pattern, in which a mutation happens in only one copy of the gene. This means that a parent with a gene mutation may pass along a copy of their normal gene or a copy of the gene with the mutation. Therefore, a child who has a parent with a mutation has a 50% chance of inheriting that mutation. A brother, sister, or parent of a person who has a mutation also has a 50% chance of having the same mutation.

How common is VHL?

It is estimated that about one in 30,000 people has VHL. About 20% of people with VHL do not have any family history of the condition. They have a de novo (new) mutation in the VHL gene.

How is VHL diagnosed?

VHL is suspected when a person has:

Multiple hemangioblastomas (blood vessel tumors) of the brain, spinal cord, or eye, or

One hemangioblastoma and kidney cysts, pancreatic cysts, pheochromocytoma, or kidney cancer, or

In young patients, VHL is also suspected with multiple bilateral clear cell renal cell carcinoma.

If a person has a family history of VHL, he or she is suspected of also having VHL if the person has any one symptom, such as hemangioblastoma, kidney or pancreatic cysts, pheochromocytoma, or kidney cancer. Genetic testing for mutations in the VHL gene is available for people suspected to have VHL. Nearly all people with VHL will be found to have the genetic mutation once tested.

What are the estimated cancer risks associated with VHL?

The risk of kidney cancer in families with VHL is estimated to be about 40%.

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