Kidney cancer (also known as renal cell cancer) occurs when malignant tumors develop in one or both kidneys. In many cases, environmental and lifestyle factors may contribute to this type of cancer, including smoking, high blood pressure, and obesity.

But it’s also possible to inherit certain genes that may increase your risk of developing kidney cancer. Unlike acquired gene mutations, inherited mutations run in families, and are dominant mutations.

Still, the overall incidence of hereditary kidney cancer is rare. An estimated 5 to 8 percentTrusted Source of all cases of kidney cancer are attributed to hereditary syndromes. Kidney cancer isn’t typically passed down from parents to children, but cancer may affect multiple generations in your family.

Having a genetic predisposition for kidney cancer doesn’t mean you’ll automatically develop it. But it’s important to know the risks associated with different inherited gene mutations.

Genetic-based kidney cancer is linked to several hereditary (family) syndromes. These cause genes to mutate, or change. While genetic mutations may be passed from parent to child, kidney cancer itself is not.

If you have any of the below inherited syndromes, you may have a higher risk of developing kidney cancer. Your doctor will likely want to regularly screen you for cancer to potentially diagnose it in the earliest stages possible.

  • Von Hippel-Lindau syndrome. Known for causing mutations in the VHL gene, von Hippel-Lindau syndrome may increase your risk for developing clear-cell renal cell carcinoma (RCC) as well as renal cysts. This syndrome causes more slow-growing cancerTrusted Source that tends to affect people at a younger ageTrusted Source. It may also lead to the development of benign (noncancerous) tumors throughout the body.
  • Birt-Hogg-Dubé syndrome. BHD syndrome is attributedTrusted Source to FLCN mutations. It can lead to the development of a variety of benign and malignant tumors throughout the body. These include kidney, skin, and lung tumors. Kidney cancer caused by BHD syndrome is slow-growingTrusted Source.
  • Hereditary papillary renal cell carcinoma. Hereditary papillary RCC is attributedTrusted Source to mutations in the MET gene. Unlike other inherited conditions that may lead to kidney cancer, this condition doesn’t cause the development of tumors in other areas of the body. It’s also a slower-growingTrusted Source cancer.
  • Hereditary leiomyoma-renal cell carcinoma. People with hereditary leiomyoma RCC have a higher risk of developing aggressiveTrusted Source papillary RCC. This condition primarily causes fibroidsTrusted Source (leiomyomas) in the skin, and the uterus may also be affected. This syndrome is linked to FH gene mutations.
  • Hereditary paraganglioma-pheochromocytoma syndromes. Also known as “familial renal cancer,” these syndromesTrusted Source develop from changes in the SDHB and SDHD genes. People with these inherited gene changes may develop kidney cancer before the age of 40Trusted Source. You may also be at a higher risk of developing paraganglioma tumors in the thyroid gland, head, and neck.
  • BAP1 tumor predisposition syndrome. This syndrome is linked to changes in BAP1 genes, and is associatedTrusted Source with clear-cell RCC. It may also increase your risk for other types of cancers, including melanoma, uveal (eye) melanoma, and mesothelioma.
  • Chromosome 3 translocation. Attributed to MITF gene mutations, this syndrome may increase your riskTrusted Source for developing clear-cell RCC.
  • Tuberous sclerosis complex. This inherited syndrome is attributed to TSC1 and TSC2 gene mutations. Most tumors from tuberous sclerosis complex are benign, and may affect your kidneys, skin, heart, and other organs. However, some kidney tumorsTrusted Source from this syndrome are clear-cell RCC.
  • PTEN hamartoma syndrome. Also known as “Cowden syndrome,” this inherited condition involves mutations in the PTEN gene. With this gene mutation, you may be at a higher riskTrusted Source of developing kidney, breast, and thyroid cancer.
  • Other genetic mutations. Other potential genetic risk factors for kidney cancer may be associated with mutations in PBRM1 and CDKN2B genes. Researchers have found that up to 40 percentTrusted Source of sporadic RCC may be linked to PBRM1, with CDKN2B being found in other cases.

Inherited kidney cancer is typically diagnosed at a younger ageTrusted Source than non-inherited (“sporadic”) cancer. About 70 percentTrusted Source of cases of inherited kidney cancer occur before the age of 46. This is different than sporadic cancers, which typically develop in your 40s, 50s, or 60sTrusted Source.

The cancer itself may also require different treatments than non-inherited kidney cancer, with some family syndromes leading to more aggressive cases. Some family syndromes also affect both in a pair of organsTrusted Source. In the case of inherited kidney cancer, it’s possible for both kidneys to have tumors.

Also, some inherited cancers may develop in multiple family members, especially in the same side of the family. If you have any of the above hereditary syndromes, you may also be at an increased riskTrusted Source of developing other types of cancers besides RCC.

ComorbiditiesTrusted Source, such as high blood pressure and smoking, may also increase your risk of developing kidney cancer.

Inherited kidney cancer is diagnosed similarly to sporadic cases of this cancer, except that you will also require genetic testing.

Besides a genetic screening, you can expect a combination of the following steps as part of a kidney cancer diagnosisTrusted Source:

Having an inherited gene mutation doesn’t mean you’ll definitely develop kidney cancer. If kidney cancer runs in your family, your doctor may recommend genetic testing from a genetic specialist to see if you have any related mutations.

Genetic testing itself won’t prevent inherited kidney cancer, but it can provide insights into your personal risk factors so that your doctor can conduct regular screenings. Early cancer detection can ensure that you receive prompt treatment for the best possible outcomes.

It’s also possible to develop kidney cancer before knowing if you have hereditary syndromes that might contribute to its development. In such cases, your doctor may refer you to genetic testing ifTrusted Source:

  • the cancer runs in your family
  • you develop kidney cancer at a young age
  • the cancer is in both kidneys
  • you have multiple tumors

Additionally, depending on the type of hereditary syndrome you have, your doctor may recommend regular screening for other types of cancer, too.

While you can’t prevent cancer that develops from inherited syndromes specifically, you may be able to reduce your riskTrusted Source of sporadic cancer. Risk factorsTrusted Source for kidney cancer include:

  • smoking
  • high blood pressure
  • overweight and obesity
  • long-term use of over-the-counter or prescription pain medications

Treatment for inherited kidney cancer depends on the stage of the cancer and whether the family syndrome you have is known to cause more aggressive or slow-growing cancers.

In most casesTrusted Source, doctors can remove kidney tumors with surgery. Kidney removal is also a possible option if you have multiple tumors in one kidneyTrusted Source. Despite surgical removal, there’s a chance that the cancer may recur.

Other likely treatments for kidney cancer includeTrusted Source:

  • immunotherapy drugs to stimulate your immune system to fight cancer cells on its own
  • targeted therapies to destroy cancer cells while preserving healthy ones (used in advanced RCC)

Rare treatment options include:

  • cyroblation to freeze and destroy smaller tumors, especially if surgery isn’t an option
  • radiofrequency ablation to destroy smaller tumors using high-energy radio waves
  • arterial embolization to shrink tumors that can’t be removed
  • radiation therapy, which is very rare, and only used if you’re not healthy enough for surgery, or only have one kidney

Chemotherapy is not an effective treatment for kidney cancer. But it may be usedTrusted Source if other therapies haven’t worked or if the cancer has spread.

Below is a list of possible questions you can ask your doctor or genetics specialist about inherited kidney cancer:

  • How often should I be screened for kidney cancer? Are risks involved?
  • What signs of kidney cancer should I look out for?
  • If I developed kidney cancer as a young adult, does that mean I need genetic testing? Should my children undergo genetic testing, too?
  • What genetic mutations did I test positive for?
  • Is my case slow-growing or aggressive?
  • Will I need surgery to treat kidney cancer? What other treatment options are available to me?
  • Will I need to worry about developing other cancers besides those that affect my kidneys?
  • What diet or lifestyle changes can I make to reduce my overall risk of cancer?

While medication use, smoking, and certain underlying conditions may increase your lifetime risk of kidney cancer, hereditary syndromes may lead to the development of cancer due to genetic mutations.

You can’t change your genes, but you can obtain regular screening to help catch possible inherited kidney cancer early. The sooner kidney cancer is detected, the more effective treatment options you may have.