Overview
Hereditary clear cell renal cell carcinoma (also called hereditary ccRCC) is an inherited form of kidney cancer. 'Clear cell' refers to how the cancer cells look under a microscope — they appear pale or clear because of the way they store certain substances. This type of cancer starts in the lining of the small tubes inside the kidney that help filter blood and make urine. Unlike most kidney cancers that happen by chance, hereditary ccRCC is passed down through families due to changes (mutations) in specific genes. The kidneys are two bean-shaped organs that sit on either side of your spine. Their main job is to clean your blood, balance fluids, and make urine. When cancer develops in the kidney, it can grow silently for a long time before causing noticeable symptoms. Common signs include blood in the urine, a lump or pain in the side or back, unexplained weight loss, and fatigue. Because this is a hereditary condition, people may develop tumors at a younger age than usual, and sometimes in both kidneys. Treatment depends on the size and spread of the cancer. Options include surgery to remove the tumor or kidney, targeted drug therapies, immunotherapy, and in some cases active surveillance for small tumors. People with hereditary ccRCC need lifelong monitoring because new tumors can develop over time. Genetic counseling is an important part of care, both for the person diagnosed and for their family members who may also carry the gene change.
Key symptoms:
Blood in the urine (urine may look pink, red, or cola-colored)A lump or mass felt in the side, back, or abdomenPersistent pain in the side or lower back not caused by injuryUnexplained weight lossFatigue and feeling unusually tiredFever that keeps coming back without an obvious causeHigh blood pressure that is hard to controlSwelling in the legs or anklesLoss of appetiteAnemia (low red blood cell count) causing paleness and weaknessTumors in both kidneys (bilateral tumors)Multiple tumors within the same kidney
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
1 eventNational Cancer Institute (NCI) — PHASE2
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Hereditary clear cell renal cell carcinoma.
1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Hereditary clear cell renal cell carcinoma.
Community
No community posts yet. Be the first to share your experience with Hereditary clear cell renal cell carcinoma.
Start the conversation →Latest news about Hereditary clear cell renal cell carcinoma
Disease timeline:
New recruiting trial: Testing of Bevacizumab, Erlotinib, and Atezolizumab in Combination for Advanced-Stage Kidney Cancer
A new clinical trial is recruiting patients for Hereditary clear cell renal cell carcinoma
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Which gene mutation do I have, and what does that mean for my cancer risk and my family members?,How often do I need imaging scans, and which type of scan is best for monitoring my kidneys?,Should my children or siblings be tested for this gene mutation, and at what age?,What is the best treatment approach for my current tumors — surgery, ablation, or watchful waiting?,Are there clinical trials I should consider for my specific gene mutation?,How can I protect my remaining kidney function over the long term?,Should I be seen at a specialized hereditary kidney cancer center, and can you refer me to one?
Common questions about Hereditary clear cell renal cell carcinoma
What is Hereditary clear cell renal cell carcinoma?
Hereditary clear cell renal cell carcinoma (also called hereditary ccRCC) is an inherited form of kidney cancer. 'Clear cell' refers to how the cancer cells look under a microscope — they appear pale or clear because of the way they store certain substances. This type of cancer starts in the lining of the small tubes inside the kidney that help filter blood and make urine. Unlike most kidney cancers that happen by chance, hereditary ccRCC is passed down through families due to changes (mutations) in specific genes. The kidneys are two bean-shaped organs that sit on either side of your spine.
How is Hereditary clear cell renal cell carcinoma inherited?
Hereditary clear cell renal cell carcinoma follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Hereditary clear cell renal cell carcinoma typically begin?
Typical onset of Hereditary clear cell renal cell carcinoma is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Hereditary clear cell renal cell carcinoma?
Yes — 1 recruiting clinical trial is currently listed for Hereditary clear cell renal cell carcinoma on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Hereditary clear cell renal cell carcinoma?
18 specialists and care centers treating Hereditary clear cell renal cell carcinoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.