Hereditary Papillary Renal Carcinoma Syndrome (HPRC)
People with hereditary papillary renal carcinoma syndrome (HPRC) have a much higher risk of developing kidney cancer.
- What is hereditary papillary renal carcinoma syndrome?
- What causes hereditary papillary renal carcinoma syndrome?
- Signs and symptoms of HPRC
- How is hereditary papillary renal carcinoma syndrome diagnosed?
- How is hereditary papillary renal carcinoma syndrome managed and treated?
- Questions to ask your healthcare team
What is hereditary papillary renal carcinoma syndrome?
HPRC is a rare hereditary condition that increases your risk of a type of kidney cancer called papillary renal cell carcinoma. People with HPRC can develop multiple papillary renal cell carcinomas in one or both kidneys.
Both men and women can have HPRC. People with HPRC may develop kidney cancer at younger ages. Nearly everyone with HPRC develops papillary renal cell carcinoma during their lifetime.
No other cancers or tumors are known to be associated with HPRC.
What causes hereditary papillary renal carcinoma syndrome?
HPRC is caused by a change (mutation) in the MET gene. HPRC happens when one of the following occurs:
- A person inherits the gene change from one parent.
- A normal gene changes by chance. When this happens, it is before birth and early in development.
The MET gene is a proto-oncogene. It normally controls how cells grow and survive. When it is mutated, cells can grow and divide without stopping.
This leads to papillary renal carcinoma tumors.
Signs and symptoms of HPRC
The only symptoms of HPRC are those caused by kidney cancers, such as:
- Blood in the urine
- Back pain
- Fullness or a lump in the belly
Some kidney cancers do not cause any symptoms. But if you are diagnosed with HPRC, there might be ways to catch papillary renal cell carcinoma early, before symptoms develop.
How is hereditary papillary renal carcinoma syndrome diagnosed?
HPRC is diagnosed by looking for MET gene changes in people with a personal or family history of papillary renal cell carcinoma. This is done with a blood test, saliva sample, or cheek swab.
Your healthcare team might recommend genetic testing if you have:
- A family history of HPRC
- A blood relative who tested positive for MET gene changes
- A personal history of more than one papillary renal carcinoma or papillary renal carcinoma before age 45
If testing shows you have a MET gene change, your children and other blood relatives might be offered testing to find out if they carry the same gene change.
For more information, see Genetic Testing for Cancer Risk.
How is hereditary papillary renal carcinoma syndrome managed and treated?
If you have HPRC, your healthcare team might recommend regular imaging tests to find kidney cancers early, before you have symptoms.
Managing HPRC syndrome
Ask your healthcare team if screening for kidney cancer is right for you.
Some doctors recommend that people with HPRC get imaging tests at regular intervals. Imaging tests might include MRI, ultrasound, or CT scans. These tests will help monitor how fast kidney tumors are growing and catch new ones when they form.
The type of testing you get, and how often you are tested, is a decision that should be made with your healthcare team. It will be based on your personal history, whether you have kidney tumors, and how fast they appear to be growing.
Treatment
HPRC itself cannot be treated, but kidney cancers can be treated when they develop. Treatment of papillary renal cell carcinoma in people with HPRC depends on the location and size of the tumors.
Localized disease
Localized papillary renal cell carcinoma is kidney cancer that is found only in the kidneys.
- Small tumors (less than 3 cm): In people with HPRC, these tumors can usually be watched with imaging tests. They may not need to be removed right away.
- Large tumors (3 cm or larger): Most experts recommend that larger tumors in people with HPRC should be removed with nephron-sparing surgery. This is also called a partial nephrectomy.
Because people with HPRC often develop more than one kidney cancer during their life, doctors try to avoid removing the kidney completely when possible.
Removing these larger tumors might prevent the cancer from spreading outside the kidneys to other parts of the body. Cancer that spreads outside the kidneys can be more difficult to treat.
Advanced or metastatic disease
If papillary renal cell carcinoma spreads beyond the kidney and cannot be removed with surgery, it cannot be cured. But it might be treated with immunotherapy or targeted therapy to shrink tumors or slow their growth.
Clinical trials are looking at treating this type of kidney cancer with medicines that target MET pathways. These medicines include savolitinib and foretinib. Talk to your care team about whether a clinical trial might be right for you.
Questions to ask your healthcare team
- What is my risk of developing kidney cancer?
- What can I do to lower this risk?
- What cancer screening tests should I get?
- Does my family history increase my risk of developing cancer?
- Should I consider genetic testing?
- If HPRC runs in my family, what are the chances my child will have it?
- Should I speak with a genetic counselor or other genetics specialist? Can you refer me to one?
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- References
Developed by the American Cancer Society medical and editorial content team with medical review and contribution by the American Society of Clinical Oncology (ASCO).
Albiges L, Gurney H, Atduev V, et al. Pembrolizumab plus lenvatinib as first-line therapy for advanced non-clear-cell renal cell carcinoma (KEYNOTE-B61): a single-arm, multicentre, phase 2 trial. Lancet Oncol. 2023;24(8):881-891.
Choueiri TK, Shuch B. Hereditary kidney cancer syndromes. UpToDate. 2026. Accessed at https://www.uptodate.com/contents/hereditary-kidney-cancer-syndromes on July 27, 2026.
Fitzgerald KN, Lee CH, Voss MH, et al. Cabozantinib Plus Nivolumab in Patients with Non-Clear Cell Renal Cell Carcinoma: Updated Results from a Phase 2 Trial. Eur Urol. 2024;86(2):90-94.
Herring JC, Enquist EG, Chernoff A, Linehan WM, Choyke PL, Walther MM. Parenchymal sparing surgery in patients with hereditary renal cell carcinoma: 10-year experience. J Urol. 2001;165(3):777-781.
Jacoba IM, Lu Z. Hereditary papillary renal cell carcinoma. Semin Diagn Pathol. 2024;41(1):28-31.
National Cancer Institute. Hereditary Papillary Renal Carcinoma (PDQ). Accessed at https://www.cancer.gov/publications/pdq/information-summaries/genetics/hprc-syndrome-hp-pdq on July 27, 2026.
Schöffski P, Wozniak A, Escudier B, et al. Crizotinib achieves long-lasting disease control in advanced papillary renal-cell carcinoma type 1 patients with MET mutations or amplification. EORTC 90101 CREATE trial. Eur J Cancer. 2017;87:147-163.
Tykodi SS, Gordan LN, Alter RS, et al. Safety and efficacy of nivolumab plus ipilimumab in patients with advanced non-clear cell renal cell carcinoma: results from the phase 3b/4 CheckMate 920 trial. J Immunother Cancer. 2022;10(2):e003844.
Yanus GA, Kuligina ES, Imyanitov EN. Hereditary Renal Cancer Syndromes. Med Sci (Basel). 2024;12(1):12. Published 2024 Feb 18.
Last Revised: October 28, 2026
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