Children's Hospital Colorado
Urologic Tumor Program

Renal Cell Carcinoma in Children

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What is renal cell carcinoma?

Renal cell carcinoma, also called hypernephroma or renal adenocarcinoma, is a fast-growing kidney cancer that can often spread to other parts of the body and organs. While renal cell carcinoma (RCC) is the most common type of kidney cancer found in adults, it rarely occurs in children. Because the disease is so common in adults but very rare in children, we make the distinction of calling it "childhood renal cell carcinoma" or "pediatric renal cell carcinoma."

The kidneys' job is to filter blood in order to remove waste and put nutrients back into the body. Long, thin tubules within the kidney help to filter out waste and reabsorb helpful substances like calcium and sodium. It is within the lining of these tubules that renal cell carcinoma starts to form. The tumor often starts in one kidney and can then spread to the other kidney and other parts of the body such as the lungs, lymph nodes and liver.

At Children's Hospital Colorado, experts in our Urologic Tumor Program have extensive experience managing all forms of pediatric kidney cancer, including childhood renal cell carcinoma.

What causes renal cell carcinoma in children?

Some evidence shows that RCC can be hereditary (passed through the genes from a parent or relative to a child); however, in most cases, it is not inherited. As the kidney cells are forming in the fetus, some of the cells do not grow properly and form a tumor or create conditions in which a tumor can form later. There is no known cause for this. In rare cases, prior exposure to chemotherapy or radiation for other tumors may cause childhood RCC as a secondary cancer.

Who gets renal cell carcinoma?

Renal cell carcinoma accounts for approximately 4% of all new kidney tumors in children and affects boys and girls the same. RCC can also be associated with the following existing conditions:

  • Von Hippel-Lindau (VHL) syndrome: VHL syndrome is a rare genetic disorder characterized by cysts and tumors throughout the body that can become cancerous. Having VHL syndrome increases the risk for renal cell carcinoma. VHL also increases the risk of developing pheochromocytomas, a rare tumor that grows on the adrenal gland.
  • Tuberous sclerosis (TSC): TSC is a rare genetic disease that can cause noncancerous tumors to grow in the brain, kidneys, heart, liver, eyes, lungs and skin. Symptoms may include seizures, intellectual disability, developmental delay, behavioral problems, skin abnormalities, and lung and kidney disease.
  • Sickle cell disease: Sickle cell disease affects the red blood cells, causing them to take the shape of a crescent or sickle instead of the disc shape. The difference in shape can cause the cells get stuck together easily and block small blood vessels, leading to organ damage and other complications.

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