Children's Hospital Colorado

Childhood Renal Cell Carcinoma

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.

What are the signs and symptoms of renal cell carcinoma in kids?

Children with RCC may experience the following symptoms:

  • Hematuria (blood in the urine)
  • A lump or mass in the abdomen or back
  • Pain in the abdomen or back that does not go away
  • Fever
  • Weight loss

How do we make a diagnosis for renal cell carcinoma?

We start with a physical examination of your child to see if they are showing any obvious signs or symptoms such as an abdominal mass or abdominal swelling. This will be followed by blood and urine tests. These tests will inform your doctor of how well the kidneys are functioning and help them decide next steps.

If signs point to a possible tumor, your doctor will schedule an image to be taken of the abdomen. There are several types of imaging tests that show various levels of detail and different layers of the anatomy. These tests can include an ultrasound, a computerized tomography (CT) scan or magnetic resonance imaging (MRI).

If the images show a tumor, your child’s doctor will most likely schedule a time to surgically remove the tumor. Specialized doctors called pathologists will examine the tissue under a microscope to confirm the diagnosis.

At Children’s Colorado, our radiology technicians are experts at taking ultrasounds, CT scans and MRIs in children to help detect tumors associated with renal cell carcinoma. We are able to complete all the tests and scans in one day. Additionally, our team of child life specialists can help patients understand their medical tests and help ease stress or anxiety.

How is childhood renal cell carcinoma treated?

Treating renal cell carcinoma in children almost always involves surgically removing the kidney tumor and the lymph nodes around the kidney. In some cases, treatment will require the compete removal of the kidney, called a radical nephrectomy.

If tests show the tumor has spread outside the kidney, we will recommend additional surgery or medication to treat those areas. Radiation therapy may also be used in rare cases.

Why choose us for the treatment of your child’s renal cell carcinoma?

At Children’s Colorado, experts in our Urologic Tumor Program have experience with all phases of childhood RCC and can provide care for patients in all stages of the disease. Our Program includes Nicholas Cost, MD, one of the few pediatric urologists who has also completed a fellowship in pediatric urologic oncology, providing a level of experience that many other programs cannot offer. Dr. Cost and the urology team continues to actively search for innovative new ways to treat RCC through RCC research and clinical trials.This means we have access to the most advanced treatments available in the field.

We also offer minimally invasive surgery (laparoscopic and robotic-assisted surgery) in select cases, which ensures the highest precision possible. Minimally invasive surgery also means shorter recovery times for patients, helping children and parents get back to their normal lives sooner.

Helpful renal cell carcinoma resources

We recommend the following resources for research and information about RCC:

  • The Children’s Oncology Group: A National Cancer Institute-supported clinical trials group and the world’s largest organization devoted exclusively to childhood and adolescent cancer research
  • CureSearch for Children’s Cancer: A nonprofit foundation working to find and accelerate cures for childhood cancer
  • The National Cancer Institute: The U.S. government’s principal agency for cancer research, providing the most recent advances in cancer and cancer research
PRODWEBSERVER2