Hereditary Kidney Cancer
Some people have certain genetic conditions that increase their risk of developing kidney cancer. These genetic conditions are most often caused by changes (mutations) in DNA that are passed down through genes inherited from a parent. It can also happen randomly for reasons doctors don’t understand.
These genetic conditions include:
- Von Hippel-Lindau (VHL) syndrome – in which cancerous and non-cancerous (benign) tumors and cysts grow in certain parts of the body, including the kidneys. People with VHL have a 30-50% (30-50 in 100) chance of developing kidney cancer in their lifetime.
- Birt-Hogg-Dube (BHD) syndrome – in which benign (non-cancer) skin tumors grow on the face, neck, and upper chest. It may also affect the lungs, causing the lung to collapse. People with BHD have about a 15-30% (15-30 in 100) chance of developing kidney tumors in their lifetime.
- Hereditary leiomyomatosis and renal cell cancer (HLRCC) – in which benign tumors grow on the skin and sometimes the uterus. About 10-16% of people (10-16 in 100) with HLRCC develop kidney cancer.
- Hereditary papillary renal cell carcinoma (HPRCC) – a rare condition in which people can develop dozens of papillary renal cancers. Nearly everyone with this condition will develop a kidney tumor.
- Tuberous sclerosis – in which tumors grow in the brain and other organs, including the kidneys. People with tuberous sclerosis very often get benign (non-cancerous) kidney tumors that can bleed if they become large. They may also have a higher chance of developing kidney cancer.
These conditions aren’t very common and make up a small number of the total number kidney cancer cases. It’s estimated that less than 5% (5 in 100) of kidney cancers are related to these genetic conditions.
Genetic testing
Genetic testing looks for certain mutations (changes) in a person’s genes. This testing uses a sample of your saliva (spit) or blood to look for gene mutations that are linked to cancer, such as the conditions listed above.
For example, VHL syndrome is caused by mutations to the VHL gene. A genetic test would look at the VHL gene and see if these mutations are present.
Genetic testing for kidney cancer may help you learn:
- If you have inherited a genetic condition that gives you a chance of certain kinds of cancer.
- If you have a high chance of developing other cancers or if it caused a separate cancer that you may have been diagnosed with. Some genetic conditions can cause multiple tumors in different parts of the body.
- If you need additional screening tests to look for cancer early. For example, if you and your doctors know you have VHL syndrome, they may recommend screening to look for tumors when they’re smaller and easier to treat.
- If you have a genetic condition that you may pass on to your children. This can help you prepare for your children’s healthcare in the future.
Your doctor will determine if you need genetic testing and what tests may be right for you.
Not everyone needs genetic testing for cancer. It’s usually done if you have a strong personal or family history of cancer, especially if it may suggest a predisposition (strong warning) for kidney cancer.
Your doctor may recommend genetic testing if you have:
- One or more relatives who had kidney cancer or other types of cancer.
- Kidney cancer at a young age
- Multiple tumors in 1 kidney, or cancer in both kidneys
- A type of kidney cancer that is commonly inherited (based on your pathology report)
If you want to get genetic testing, your healthcare team may recommend you get genetic counseling. Genetic counseling is when you talk with a specially trained health provider about your chance of a genetic disease. They can help you understand:
- Your personal and family medical history
- A map called a pedigree will be made to visualize your family medical history by generation, similar to a family tree
- Your options for genetic testing
- The risks and benefits of genetic testing
- The results of genetic testing and how to manage the results

A pedigree shows the pattern of cancer and diagnosis of cancer in your family. It can help you and your doctors make choices about getting genetic testing or screenings.
References:
Information on this page last reviewed: January, 2025
Keep Learning:
The Kidney Cancer Association provides educational literature for anyone impacted by kidney cancer.