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Renal Cell Carcinoma Facts

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What is Renal Cell Carcinoma of the kidney?

Renal cell carcinoma (RCC) is a very rare cancer in children with only about 2 to 5 cases diagnosed in the United States per year. Up until now no large studies have been performed in children because it is so rare. Therefore, there is limited information available on the biology, treatment, and outcome for renal cell carcinoma in children.

There are 4 subtypes of renal cell carcinoma. Most of the subtypes are seen in adults, but rarely in children. Just remember that adult renal cell carcinoma is very different from renal cell carcinoma in children, so information obtained on adult tumors can not be applied to children. In children the tumors act differently, have different genetic changes, and appear to need different treatments.

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What are the major subtypes of Renal Cell Carcinoma?

  • Papillary renal cell carcinoma
  • Renal medullary carcinoma
  • Renal cell carcinoma with TFE3 or TFEB (gene mutations)
  • Renal cell carcinoma, clear cell subtype

For more information about the in-depth biology of these subtypes.

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How is Renal Cell Carcinoma diagnosed?

The first test used to diagnose renal cell carcinoma is often an abdominal ultrasound. A specialized ultrasound called a Doppler ultrasound is done to evaluate if the tumor has grown into the blood vessels. This is followed by a CT scan and/or MRI scan of the abdomen and pelvis to further evaluate the mass.

In addition, several imaging studies are used to make sure the tumor has not spread to other organs. A CT scan of the lung allows a doctor to see if the mass in the kidney has spread to the lung which is the most common site for this type of tumor to spread. Chest x-rays (CXR) are often done in addition to CT scans, and are used mostly during and after treatment. A MRI or CT scan of the brain is done to check and see whether or not the renal cell carcinoma has spread to or started forming in the brain. A bone scan should be done, since the bone is also a common site for renal cell carcinoma to spread. A PET scan may be done, if it is available and if your child can hold still long enough for it.

It is recommended that as many of the above imaging studies as possible be done at the hospital where your child will be treated.

Once the diagnosis of renal cell carcinoma has been confirmed by a pathologist, the oncologist will evaluate the results and assign a stage to the tumor. Staging is a process that determines how widespread the cancer may be. The stage tells the doctor if the cancer has spread and if so, how far it has spread. The stage of the tumor will determine what type of treatment your child will receive.

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How is Renal Cell Carcinoma treated?

Not much is known about the treatment of renal cell carcinoma in children; however, chemotherapy and radiation therapy have not been found to be useful in adults and are very unlikely to be useful in children.

Therefore, surgery is the treatment of choice. It is important for surgery to be done by a pediatric surgeon who is used to taking care of children with cancer. The surgeon should try to remove all of the tumor, and if possible, remove all of the metastases.

For children in stage I, II, or III, no additional treatment beyond surgery is recommended as long as all of the tumor was surgically removed. This is because stage I, II, and III tumors are usually localized, and localized tumors tend to have relatively good responses to surgical removal.

For children with stage IV, it is advised to try to remove all of the tumor, as long as this can safely be done. Treatment with immunotherapy such as interferon alpha and interleukin-2 or experimental clinical phase I or II studies are recommended. Numerous studies have demonstrated response rates of 10-20% with immunotherapy.

If imaging studies show that a tumor cannot be completely removed, a biopsy of the tumor is done. The biopsy is done to obtain tissue, confirm that the tumor is renal cell, and then to determine the subtype as well as the characteristics of the tumor.

More information about staging.

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What is being done to help better treat Renal Cell Carcinoma?

For the first time, the Children's Oncology Group is performing a widely available study of renal tumors that includes this rare type of tumor in children. This study will help researchers understand more about how renal cell carcinoma in children is different from adult renal cell carcinoma, and will help researchers come up with a more specific treatment to better treat renal cell carcinoma in children. Your child can participate in this study by having his or her doctor send small pieces of their tumor to the Children's Oncology Group. Tumor tissue is generally taken at the time of surgery (only tissue that is not needed for pathologic evaluation or specific care of your child is used). A small sample of blood and urine should be sent to the Children's Oncology Group as well.

More information about this clinical trial.

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What is the prognosis for survival?

As in most cancer, survival rates for renal cell carcinoma depends on the stage of the tumor. Children with:

  • Stage I: > 90% survival
  • Stage II: > 80% survival
  • Stage III: > 70 % survival
  • Stage IV: < 15 % survival

The most important factor for longer survival is the removal of all possible tumors. This means that a stage III child with all of the tumor removed will be expected to do better than a stage III child where some of the tumor had to be left behind. A stage IV child with all visible tumor removed will do better than any other staged child in which the tumor had to be left behind in his or her body.

Written and reviewed by: Susanne Ragg, MD, PhD; Deannie Lee, MD, PhD; Mary M Davis MD; James Hill, MD; Mercy Odueyungbo, B.S.; Ann Haddix, RN, MSN, CPNP; Jada Paine, MS; Julie Haydon, BS; Michelle Lucke, MPH; Elaine Skopelja, MALS, AHIP; Kellie Kaneshiro, AMLS, AHIP. Amber McMahon, B.S. Date last reviewed: August 2006.

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