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MyPART - My Pediatric and Adult Rare Tumor Network
 

Papillary Renal Cell Carcinoma

What is papillary renal cell carcinoma?

Papillary renal cell carcinoma, or PRCC, is a type of kidney cancer. The kidneys work by removing waste products from the blood. Papillary renal cell carcinoma is a cancer of the tubes that filter those waste products from the blood. There are two types of papillary renal cell carcinoma. Type 1 is more common and grows slowly. Type 2 is more aggressive and grows more quickly. 

How common is PRCC?

PRCC makes up about 15% of all renal cell carcinoma, which is the most common type of kidney cancer. 

How is PRCC diagnosed?

Some people have symptoms with PRCC, but others don’t. Symptoms can include:

  • Blood in the urine
  • Pain
  • Weight loss
  • Feeling tired
  • Fever
  • A lump in the side

Sometimes, papillary renal cell carcinoma is found in a patient accidentally while performing a physical exam or a scan for something else. 

Imaging: If you have symptoms of PRCC, your doctor will use imaging scans such as CT, MRI, and ultrasound to look at where the PRCC tumor is in the kidney and how big it is. They will also check for signs that the tumor has spread to other parts of the body. 

Biopsy: To check if the tumor is PRCC, your doctor will do a biopsy, taking a small sample from the tumor with a needle. An expert, called a pathologist, will study the cells from the sample under the microscope to see what kind of tumor it is. 

How is PRCC treated?

Treatment for papillary renal cell carcinoma for each patient is unique. You should go to an expert in PRCC treatment to decide the best approach for your tumor. You can contact MyPART for help fining experts near you. 

Surgery: Once papillary renal cell carcinoma is diagnosed, you may have surgery to remove the tumors from the kidney. In later stages, surgery may be difficult, in which case your doctor will discuss othe options with you.

Radiation Therapy: The radiation is aimed at the tumor area to kill the tumor cells and to prevent it from growing back.

Chemotherapy: When surgery is not possible or when the cancer has spread, chemotherapy may be used to treat PRCC, but it is not used often.

Does PRCC run in families?

PRCC usually occurs randomly, which is called sporadic papillary renal cell carcinoma.

PRCC can also run in families, but this is rare. One form of PRCC that runs in families is called hereditary papillary renal cell carcinoma. Hereditary papillary renal cell carcinoma usually has a mutation in a part of the chromosomes (the parts of your cells that contain all of your genes) called the MET gene. 

Another form of PRCC that runs in families is called Hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC). This is characterized by alterations in a gene called fumarate hydratase. Patients or affected relatives with this condition may also have skin tumors called leiomyomas and uterine fibroids, which usually happen at a young age.

How does PRCC form?

Scientists are always working to understand how cancer forms, but it can be hard to prove. We do know that patients with PRCC have many mutations in their chromosomes. This can cause the cells not to function as they should. 

What is the prognosis for someone with PRCC?

The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as

  • Where the tumor is in your body
  • If the cancer has spread to other parts of your body
  • How much of the tumor was taken out during surgery

If you want information on your prognosis, it is important to talk to your doctor. NCI also has resources to help you understand cancer prognosis

Doctors estimate survival rates by how groups of people with PRCC have done in the past. Because there are so few people with PRCC, these rates may not be very accurate. They also can’t consider newer treatments being developed.

In general, type 2 papillary renal cell carcinoma has a poorer prognosis than type 1.