Is Kidney Cancer Hereditary? Genetics, Risk, and Prevention

Understand hereditary kidney cancer: genetics, risk factors, key syndromes, prevention, and treatment.

By Sneha Tete, Integrated MA, Certified Relationship Coach

Created on Sep 25, 2025

Understand hereditary kidney cancer: genetics, risk factors, key syndromes, prevention, and treatment.

Kidney cancer is a significant health concern, with most cases arising sporadically, but a noteworthy minority linked to inherited genetic mutations and syndromes. Understanding the hereditary aspects of kidney cancer is crucial for early detection, prevention, and management, especially for individuals with a strong family history or other risk factors.

Overview: Hereditary vs. Non-Hereditary Kidney Cancer

Kidney cancer develops when abnormal cells in the kidney grow out of control, forming a tumor. While the majority of kidney cancers are sporadic, meaning they occur by chance without a known inherited genetic basis, approximately 5% to 8% of all kidney cancers are classified as hereditary or familial in origin.

Hereditary kidney cancer involves inherited mutations (or “pathogenic variants”) in specific genes that increase the lifetime risk of developing kidney cancer.
Non-hereditary kidney cancer (also called sporadic kidney cancer) typically occurs randomly, influenced by environmental exposures or other lifestyle-related factors.

Hereditary kidney cancers often exhibit certain distinguishing features:

Diagnosed at a younger age compared to sporadic cases.
May be multifocal (multiple tumors) or affect both kidneys.
Associated with other cancers or non-cancerous tumors typical of the underlying genetic syndrome.
Sometimes more or less aggressive, depending on the syndrome involved.

Who Is at Risk for Hereditary Kidney Cancer?

You might be at increased risk for hereditary kidney cancer if one or more of the following applies:

Multiple family members (especially first-degree relatives) have been diagnosed with kidney cancer.
You were diagnosed at a younger-than-usual age with kidney cancer.
There are cases of bilateral kidney tumors (tumors in both kidneys) or multiple tumors in the same kidney.
Other types of cancer cluster within your family.

Genetics Behind Hereditary Kidney Cancer

Hereditary kidney cancer is usually due to an inherited pathogenic variant (mutation) in a specific gene associated with a hereditary cancer syndrome. These syndromes follow an autosomal dominant inheritance pattern, meaning only one mutated copy of the gene (inherited from either parent) is needed to increase risk.

Scientific advances have identified up to a dozen hereditary cancer syndromes and more than ten genes that, when mutated, significantly elevate the risk of kidney cancer. However, four major hereditary syndromes account for most familial kidney cancer cases.

Major Hereditary Kidney Cancer Syndromes

Syndrome	Gene Involved	Associated Cancer Types	Other Features
Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC)	FH	Type 2 papillary renal cell carcinoma	Skin and uterine leiomyomas, paragangliomas
Von Hippel-Lindau Disease (VHL)	VHL	Clear cell renal carcinoma	Hemangioblastomas, pheochromocytomas, pancreatic cysts
Birt-Hogg-Dubé Syndrome (BHD)	FLCN	Various types of kidney cancer	Skin fibrofolliculomas, spontaneous pneumothorax
Hereditary Papillary Renal Cancer (HPRC)	MET	Type 1 papillary renal cell carcinoma	No major extra-renal features

1. Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC)

HLRCC is caused by mutations in the FH (fumarate hydratase) gene.

Leads to an increased risk of an aggressive form of kidney cancer, primarily type 2 papillary renal cell carcinoma.
Can also cause smooth muscle tumors (leiomyomas) in the skin and uterus, and sometimes benign paragangliomas.
The mutation may impair the cell’s energy production, promoting malignant transformation.
An affected parent has a 50% chance of passing the mutation to each child.

2. Von Hippel-Lindau Disease (VHL)

VHL syndrome stems from mutations in the VHL gene and is one of the most well-known inherited cancer syndromes.

Increases risk for clear cell renal carcinoma, pheochromocytomas (adrenal tumors), and other tumors.
Also involves blood vessel tumors (hemangioblastomas) and pancreatic cysts or tumors.

3. Birt-Hogg-Dubé Syndrome (BHD)

BHD arises from mutations in the FLCN (folliculin) gene.

Characterized by multiple skin fibrofolliculomas (small benign tumors) and kidney tumors.
Markedly increases risk of kidney cancer and is also linked to spontaneous pneumothorax (collapsed lung).

4. Hereditary Papillary Renal Cancer (HPRC)

HPRC is due to mutations in the MET gene.

Significantly raises risk for type 1 papillary renal cell carcinoma.
Extra-renal manifestations are rare or absent.

Additional Hereditary Syndromes

While four syndromes are most commonly discussed, research has identified other genetic syndromes that raise kidney cancer risk, albeit less frequently. These include:

Tuberous sclerosis complex
BAP1 tumor predisposition syndrome
Succinate dehydrogenase kidney cancer syndrome
Cowden syndrome and PTEN hamartoma tumor syndrome

Each of these syndromes is linked to specific gene mutations and carries unique risks for kidney and other types of cancer.

How Are Hereditary and Non-hereditary Kidney Cancers Different?

Age at diagnosis: Hereditary cases present at younger ages.
Number of tumors: Hereditary cancers may be bilateral (both kidneys) or multifocal (more than one tumor in a kidney) compared to mostly single, unilateral sporadic tumors.
Genetic features: Presence of identifiable gene mutations or syndromes in hereditary, absent in sporadic cases.
Associated risks: Hereditary syndromes may increase risk for other cancers or benign growths.
Treatment and prognosis: May differ based on the aggressiveness of the syndrome and recurrence risk.

Risk Factors for Kidney Cancer

Most people diagnosed with kidney cancer do not have an inherited syndrome. Instead, several risk factors for kidney cancer include:

Smoking
High blood pressure (hypertension)
Obesity or being overweight
Chronic use of certain pain medications (over-the-counter or prescription)
Older age (risk increases after age 50)
Male sex (men are more likely than women to develop kidney cancer)
Family history of kidney cancer (hereditary or familial risk)

Can Hereditary Kidney Cancer Be Prevented?

Although you can’t change your genetics, proactive measures can make a significant difference. Here’s what you need to know about preventing and managing hereditary kidney cancer:

Genetic Testing: Recommended if you have a suspicious family history, were diagnosed at a young age, have bilateral or multiple tumors, or have other features suggesting hereditary syndrome.
- Testing itself cannot prevent cancer, but it enables early detection via regular screening and surveillance.
Regular Screening: If a mutation is confirmed, doctors often recommend more frequent screening for early kidney tumors and sometimes for additional syndromic cancers/manifestations.
- Screening approaches may include abdominal imaging (MRI, ultrasound, or CT scans) on a regular schedule.

Reducing Risk for Sporadic Kidney Cancer

While you can’t prevent cancers caused by inherited gene mutations, you can take steps that may help reduce your overall kidney cancer risk, including that of sporadic cases:

Quit smoking.
Maintain healthy blood pressure.
Aim for a healthy weight through balanced nutrition and exercise.
Avoid long-term use of painkillers unless prescribed by your doctor.

How Is Hereditary Kidney Cancer Treated?

Management of hereditary kidney cancer generally follows similar principles as treatment for sporadic (non-hereditary) kidney cancer, but there are unique considerations:

Surgical removal (partial or complete nephrectomy) is the cornerstone when possible.
For multiple or bilateral kidney tumors, nephron-sparing surgery (preserving as much normal kidney tissue as possible) is preferred, especially in syndromic cases.
Active surveillance may be considered in specific situations (depending on tumor size, growth rate, or presence of underlying syndrome).
Immunotherapy: Uses medications that help your immune system identify and destroy cancer cells (common in advanced cases).
Targeted therapy: Advanced kidney cancers may be treated with drugs that specifically target molecules involved in the growth of cancer cells.
Ongoing monitoring and screening for associated tumors, depending on the genetic syndrome involved.

Despite optimal treatment, kidney cancer can recur. It is important for people with hereditary syndromes to receive ongoing specialist care.

Genetic Counseling: Who Should Consider It?

Consider genetic counseling and possibly testing if you or a first-degree relative have any of these:

Kidney cancer diagnosed under age 46–50, especially without known environmental risk factors.
Multiple kidney tumors, or tumors in both kidneys.
Two or more close relatives with kidney cancer.
Personal or family history of another hereditary cancer syndrome (such as VHL, BHD, or Tuberous Sclerosis).
Skin lesions, uterine fibroids, or signs suggestive of a hereditary cancer syndrome (based on your physician’s assessment).

Living with a Family History of Kidney Cancer

If kidney cancer runs in your family, take proactive steps:

Discuss your family and personal medical history with your healthcare provider.
Request referral to a genetic counselor as appropriate.
Stay up to date with screening recommendations based on your risk profile.
Adopt healthy lifestyle measures (quit smoking, exercise, manage blood pressure).

Remember: having an inherited risk does not absolutely guarantee that you will develop kidney cancer, but it does mean you should be vigilant and proactive about your health.

Frequently Asked Questions (FAQs)

Q: What percentage of kidney cancers are inherited?

A: Approximately 5–8% of kidney cancers are thought to have a hereditary basis, due to known gene mutations or inherited cancer syndromes.

Q: If I have a parent with kidney cancer, am I guaranteed to get it too?

A: No. While some genetic syndromes raise your risk, most people with a family history don’t develop kidney cancer. Your doctor may discuss genetic testing if your family history suggests a hereditary cause.

Q: Do all hereditary kidney cancers act more aggressively?

A: Not always. Some syndromes, like HLRCC, can cause aggressive cancers, while others may have slower-growing tumors. The clinical behavior depends on the specific syndrome and gene involved.

Q: Can lifestyle changes prevent hereditary kidney cancer?

A: Although you can’t change your genes, healthy habits such as quitting smoking and maintaining blood pressure can reduce your risk of sporadic cancer and support overall kidney health.

Q: How do I know if I should get genetic testing?

A: Consider testing if you have a strong family history of kidney cancer, were diagnosed at a young age, have multiple tumors, or other indicators associated with hereditary syndromes. Always consult with a healthcare provider or genetic counselor before pursuing testing.

Summary

If you or your family members have been diagnosed with kidney cancer—especially at a young age—it is important to speak with your healthcare provider about hereditary risk and consider genetic counseling. Early diagnosis, proactive surveillance, and targeted management are key strategies that can improve outcomes for individuals with inherited cancer syndromes.

References

Author

Sneha TeteBeauty & Lifestyle Writer

Sneha is a relationships and lifestyle writer with a strong foundation in applied linguistics and certified training in relationship coaching. She brings over five years of writing experience to thebridalbox, crafting thoughtful, research-driven content that empowers readers to build healthier relationships, boost emotional well-being, and embrace holistic living.

Read full bio of Sneha Tete