Overview
Inherited renal cancer-predisposing syndrome (Orphanet code 319328) is a broad term for a group of hereditary conditions that significantly raise a person's lifetime risk of developing kidney (renal) cancer. Unlike most kidney cancers that occur by chance, these syndromes are caused by changes (mutations) in specific genes that are passed down through families. Because of this, multiple family members across generations may be affected. The kidneys are two bean-shaped organs that filter waste from the blood and produce urine. In these syndromes, abnormal gene changes can cause tumors to grow in one or both kidneys, sometimes at a younger age than typical kidney cancer and sometimes as multiple tumors. Depending on the specific syndrome involved, other parts of the body may also be affected, including the eyes, skin, brain, adrenal glands, and pancreas. This category includes several well-known syndromes such as Von Hippel-Lindau (VHL) disease, hereditary papillary renal cell carcinoma (HPRC), Birt-Hogg-Dubé syndrome (BHD), hereditary leiomyomatosis and renal cell cancer (HLRCC), and succinate dehydrogenase-related renal cancer. Treatment focuses on regular surveillance to catch tumors early, surgical removal of tumors when needed, and targeted drug therapies. Early detection through genetic testing and regular monitoring can greatly improve outcomes.
Key symptoms:
One or more tumors in the kidney, sometimes in both kidneysBlood in the urine (urine appears pink, red, or brown)Pain or a lump in the side or lower backUnexplained weight lossFatigue and feeling generally unwellHigh blood pressure that is hard to controlSkin growths, bumps, or unusual skin lesions (depending on the syndrome)Cysts in the kidneys, pancreas, or liverTumors in the adrenal glands (pheochromocytoma)Eye problems such as retinal tumors or vision changesTumors in the brain or spinal cord (in VHL disease)Uterine fibroids at a young age (in HLRCC)Lung cysts or collapsed lung (in Birt-Hogg-Dubé syndrome)
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)
Treatments
No FDA-approved treatments are currently listed for Inherited renal cancer-predisposing syndrome.
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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 Inherited renal cancer-predisposing syndrome.
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
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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 specific inherited renal cancer syndrome do I have, and which gene mutation was found?,How often do I need imaging scans, and which organs need to be monitored?,At what tumor size should I consider surgery or another treatment, and what are my options?,Should my children, siblings, and parents be tested for this gene mutation?,Am I a candidate for belzutifan or any other targeted medication?,Are there any clinical trials I should know about for my specific syndrome?,What lifestyle changes, if any, can help protect my kidney function long-term?
Common questions about Inherited renal cancer-predisposing syndrome
What is Inherited renal cancer-predisposing syndrome?
Inherited renal cancer-predisposing syndrome (Orphanet code 319328) is a broad term for a group of hereditary conditions that significantly raise a person's lifetime risk of developing kidney (renal) cancer. Unlike most kidney cancers that occur by chance, these syndromes are caused by changes (mutations) in specific genes that are passed down through families. Because of this, multiple family members across generations may be affected. The kidneys are two bean-shaped organs that filter waste from the blood and produce urine. In these syndromes, abnormal gene changes can cause tumors to grow
How is Inherited renal cancer-predisposing syndrome inherited?
Inherited renal cancer-predisposing syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Inherited renal cancer-predisposing syndrome typically begin?
Typical onset of Inherited renal cancer-predisposing syndrome is adult. Age of onset can vary across affected individuals.
Which specialists treat Inherited renal cancer-predisposing syndrome?
1 specialists and care centers treating Inherited renal cancer-predisposing syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.