What to know about hereditary papillary kidney cancer

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Hereditary papillary kidney cancer is a rare genetic syndrome in which a person may have a higher than average risk of developing type one papillary kidney cancer. An individual may inherit hereditary papillary kidney cancer from one or both parents.

Papillary kidney cancer forms in the lining of the tubes inside the kidneys. These renal tubes are responsible for removing waste products from the blood and filtering them out of the body through urine. They also filter necessary fluids back into the blood.

There are two types of papillary kidney cancer. Type one papillary kidney cancer is more common and typically slow growing, meaning it is less likely to spread to other parts of the body. Type two papillary kidney cancer is typically more aggressive, meaning it is more likely to spread.

According to the National Cancer Institute, papillary kidney cancer accounts for approximately 15% of all kidney cell cancers.

Hereditary papillary kidney cancer can occur when there are harmful changes or mutations of the MET gene. The MET gene is responsible for making a type of protein that sends signals from cell to cell in the body, and helps cells grow and survive.

When there are mutations of the MET gene, atypical cells can grow. These atypical cells can develop into papillary kidney cancer and potentially spread to other parts of the body.

2024 research suggests there is a 50% chance a person will inherit mutations of the MET gene if one of their parents has hereditary papillary kidney cancer.

If an individual with hereditary papillary kidney cancer develops type one papillary kidney cancer, they may experience several symptoms. It is also possible a person may not experience any symptoms.

Symptoms of type one papillary kidney cancer can include:

• unexplained weight loss
• fatigue
• fever
• pain in the lower back or abdomen
• a lump in the side of the body
• blood in urine

An individual may want to undergo genetic testing to find out if they have hereditary papillary kidney cancer, particularly if a person has a family history of the condition.

If genetic tests reveal mutations of the MET gene are present, a doctor may recommend that a person attends regular screenings throughout their lifetime.

If an individual with hereditary papillary kidney cancer experiences symptoms of papillary kidney cancer, or a doctor has a concern following a regular screening appointment, they may carry out a physical examination. A doctor may also perform various imaging scans such as CT, MRI, and ultrasound to help identify any tumors.

If a tumor is present, a doctor may take a biopsy sample from the tumor with a needle and examine it for the presence of papillary kidney cancer.

If a person with hereditary papillary kidney cancer has a tumor, a doctor may recommend surgery to remove it. This can help prevent the cancer from spreading to other parts of the body.

A doctor may also recommend chemotherapy and radiation therapy to help kill further papillary kidney cancer cells and prevent them from growing back.

Research from 2023 suggests that alternative therapies such as targeted therapy, immunotherapy, and combination therapy may help treat papillary kidney cancer in future. However, there is limited research and clinical trials are still ongoing.

If an individual has hereditary papillary kidney cancer, the type of cancer they are more at risk of developing is type one papillary kidney cancer.

Type one papillary kidney cancer is typically slow growing, less likely to spread to other parts of the body, and therefore more likely to stay localized to the kidney.

According to the Surveillance, Epidemiology, and End Results (SEER) database, the 5-year relative survival rate following diagnosis for localized type one papillary kidney cancer is approximately 93%.

Hereditary papillary kidney cancer is a genetic syndrome that may increase the risk of a person developing type one papillary kidney cancer.

Papillary kidney cancer affects the cells in the lining of the tubes in the kidneys that filter waste from the blood through urine. Type one papillary kidney cancer is a less aggressive form of the condition.

Hereditary papillary kidney cancer can occur when someone inherits certain mutations of the MET gene from at least one of their parents.

If an individual has hereditary papillary kidney cancer, a doctor may recommend regular screenings to check for any papillary kidney cancer tumors. A doctor may use imaging scans to help diagnose any tumors.

If papillary kidney cancer tumors are present, a doctor may recommend surgery to remove the tumors, alongside chemotherapy and radiation therapy, to try and prevent the cancer cells from growing back.

A person should speak with a healthcare professional for more information on an individual basis.