Have you ever thought about what the two bean-shaped organs in your lower back do? Those are your kidneys. They play a vital role in keeping your body healthy by filtering about half a cup of blood every minute. They remove waste and extra water to produce urine while also maintaining the balance of fluids and minerals in your body.
Like other organs, the kidneys can develop cancer, although it is less common compared to cancers such as breast, lung, or colorectal cancer. In recognition of World Kidney Day, let’s take a closer look at kidney cancer, especially the types that can be inherited, and how they can be managed.
Hereditary vs Non-Hereditary Kidney Cancer
Most kidney cancers are not inherited. They usually occur due to random genetic changes that happen over time in kidney cells.
However, about 5-8% of kidney cancers are hereditary, meaning they are caused by inherited genetic mutations passed down in families. These conditions are called hereditary cancer syndromes. Since hereditary kidney cancer can often be diagnosed at an earlier age and have a higher risk of developing other types of cancer, it might need different treatments and monitoring approaches from general somatic kidney cancer.
Different Types of Hereditary Kidney Cancer
There are different types of hereditary kidney cancer, including:
- Von Hippel–Lindau (VHL) Syndrome: caused by VHL (tumor suppressor gene) mutation causing mainly slow-growing clear-cell renal cell carcinoma (RCC)
- Key features: kidney tumor; tumors in the brain, spinal cord, retina (eyes), ear, pancreas, or part of the reproductive organ.
- Hereditary Leiomyomatosis & Renal Cell Cancer (HLRCC): caused by FH (fumarase gene) mutation often appearing as single but aggressive kidney tumor
- Key features: kidney tumor, tumors in the skin or uterus in women, tumor in the adrenal glands, blood vessels or nerves
- Birt-Hogg-Dubé (BHD) Syndrome: caused by FLCN (folliculin gene) mutation often developing multiple slow growing tumors
- Key features: kidney tumor, skin tumors (fibrofolliculomas), lung cysts, collapsed lung (pneumothorax)
- Hereditary Papillary Renal Cancer (HPRC): caused by MET (cell signaling gene) mutation often only increasing slow-growing kidney cancer risk at the renal tubules that remove waste from blood and create urine
A Case Study of Two Patients Having Identical Kidney Cancer Manifestation with Two Different Genetic Testing Approach: Random vs Inherited Mutation
Imagine two patients, both 40-year-old women, who each have a 3 cm tumor in their right kidney. Both undergo the same surgery to remove the tumor.
At first glance, they appear identical. But their situations are actually very different.
- Patient A developed cancer due to a random mutation
- Patient B inherited the mutation from her family.
In the first case scenario, the surgeon sends the tumor tissue for Somatic Testing, and both reports come back identical: “VHL Mutation Detected.”
| Impact Area | Patient A (Somatic/Random) | Patient B (Germline/Inherited) |
| Diagnosis | Accurate. The doctor correctly identifies the mutation driving the tumor. | Incomplete. The doctor sees the mutation in the tumor but doesn’t know it’s in her entire body. |
| Medication | Standard post-operation monitoring. If it returns, she gets standard therapy. | Missed Opportunity. She isn’t offered VHL syndrome-specific drugs (like Belzutifan) because the systemic nature of her disease is unknown. |
| Prevention | Safe. No further action is needed for her other organs. | Catastrophic. Because her VHL syndrome is “hidden,” she isn’t screened for tumors in her brain or eyes. She risks sudden blindness or neurological damage. |
| The Family | No risk to her children. | The Tragedy. Her children are never tested. Her son might develop an advanced tumor at age 20 that could have been caught at age 10. |
In the long term, patient A will be fine although she might not know her family’s chance to develop the same cancer, while patient B missed the root cause of her kidney cancer causing fatal risks in the future which could have been prevented early.
In the second case scenario, both patients receive a Hereditary Cancer Screening through their blood sample alongside their clinical workup.
| Impact Area | Patient A (Somatic/Random) | Patient B (Germline/Inherited) |
| Diagnosis | Cleared. Her blood test is negative. The doctor confirms her mutation was a “one-off” event limited to that kidney. | Life-Saving Discovery. The blood test is positive. The doctor realizes the kidney tumor was just the first symptom of VHL Syndrome. |
| Medication | She avoids unnecessary high-tier medications and sticks to basic monitoring. | The Right Key. She is immediately eligible for Belzutifan, which shrinks the VHL tumors across her whole body without surgeries. |
| Prevention | She is discharged with a “Clean Bill of Health.” | The Proactive Shield. She begins a “Total Body Surveillance” plan (Annual MRI and Eye exams). Any new “glitch” is caught at a microscopic, treatable stage. |
| The Family | She reassures her family they aren’t at risk. | The Cycle is Broken. Her children are tested. Those who are positive start screening at age 10. They will never face the “Late Stage” diagnosis their mother did. |
When hereditary testing is performed, Patient A gains reassurance that her cancer is isolated, needing just basic monitoring and will not be inherited to her children. On the other hand, patient B gets informed about the inherited mutation which might develop other tumors in herself or her children. Hence, she can begin life-saving surveillance and systemic medication while her children can do genetic screening early.
Why Hereditary Screening Matters
- For diagnosis: It helps doctors understand whether cancer is a single event or part of a broader inherited condition.
- For treatment: It can guide the use of targeted therapies designed specifically for hereditary cancers.
- For prevention: It allows families to move from reacting to symptoms toward proactive, early detection.
NGI offers a range of hereditary cancer screening options, from targeted panels, such as the Hereditary Renal Carcinoma Panel, to comprehensive screening for both men and women of up to 25 types of cancer.
Hereditary testing is more than just identifying cancer risk; it is about giving you and your family the power to act early, monitor proactively, and prevent life-threatening complications before they happen. Don’t wait for symptoms to appear, knowing your genetic risk today can help protect your health and your family’s future tomorrow.
References:
- National Cancer Institute. “Hereditary Kidney Cancer Syndromes (PDQ®)–Patient Version – NCI.” Www.cancer.gov, 23 Aug. 2019, www.cancer.gov/types/kidney/patient/kidney-genetics-pdq.
- National Institute of Diabetes and Digestive and Kidney Diseases. “Your Kidneys & How They Work.” National Institute of Diabetes and Digestive and Kidney Diseases, June 2018, www.niddk.nih.gov/health-information/kidney-disease/kidneys-how-they-work.
- Wang, Linda. “Belzutifan Works Steadily in People with VHL-Associated Tumors | Center for Cancer Research.” Cancer.gov, 2025, ccr.cancer.gov/news/article/belzutifan-works-steadily-in-people-with-vhl-associated-tumors. Accessed 23 Feb. 2026.
