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MyPART - My Pediatric and Adult Rare Tumor Network
 

Translocation Renal Cell Carcinoma

What is translocation renal cell carcinoma?

Renal cell carcinoma is cancer that grows in the kidney. There are many different types of kidney cancer and translocation renal cell carcinoma is one subtype. Translocation renal cell carcinoma is also called Xp11.2 translocation renal cell carcinoma or TRCC. It was recognized as a type of kidney cancer in 2004. This type of cancer usually grows slowly. Patients usually do not experience symptoms.

This type of cancer is more common in children and young adults than adults. It is also slightly more common in females. However, adults with this type of cancer tend to have more aggressive or fast-growing cancer. 

How common is TRCC?

TRCC makes up only 1 to 5% of all cases of renal cell carcinoma. Out of all of the childhood cases of renal cell carcinoma, translocation renal cell carcinoma makes up about 20% of the cases.    

How is TRCC diagnosed?

Imaging: Sometimes, TRCC is discovered when imaging the body using tests like CT, PET, and MRI. More tests are needed to find out if the cancer is translocation renal cell carcinoma.

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

How is TRCC treated?

Treatment options for patients depend on the how the cancer is growing and whether the cancer has spread to other parts of the body, called metastasis.  

Surgery: For patients with less advanced stages of TRCC, surgery to remove the tumor is a treatment option.

Targeted therapy: Patients with more advanced TRCC are given specific types of drugs to kill the cancer cells.  

Does TRCC run in families?

No, TRCC does not run in families.

How does TRCC form?

We know that in TRCC, chromosomes (the parts of your cells that contain all of your genes) break apart and get put back together in the wrong way. This can cause cells to not function like they should. In translocation renal cell carcinoma, a gene called TFE3 is broken apart and rearranged. Doctors will look for this change in chromosomes to confirm that your cancer is translocation renal cell carcinoma.

What is the prognosis for someone with TRCC?

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 translocation renal cell carcinoma survival rates by how groups of people with TRCC have done in the past. Because there are so few patients, these rates may not be very accurate. 

Patients diagnosed in childhood usually have a better prognosis than patients diagnosed in adulthood. Translocation renal cell carcinoma can spread to other parts of the body many years after it is diagnosed, so it is important that patients continue to visit their doctor after treatment.