Children's Hospital Colorado

Clear Cell Sarcoma of the Kidney in Children

What is clear cell sarcoma of the kidney?

Clear cell sarcoma of the kidney (CCSK) is a form of kidney cancer that is rarely found in children. Tumors that form in or on the kidneys are also known as renal tumors. At Children's Hospital Colorado, our Urologic Tumor Program treats children with every type of renal tumor including CCSK, which represents about 4% of all pediatric renal tumors.

Considered to be more aggressive than other renal tumors, such as the Wilms tumor, clear cell sarcoma of the kidney has a tendency to metastasize, or spread, to the bones and other organs. CCSK tumors also have a higher chance of returning compared to other pediatric renal cancers.

What causes clear cell sarcoma of the kidney?

Clear cell sarcoma of the kidney is not thought to be hereditary, meaning a parent or relative cannot pass clear cell sarcoma to a child. While there is no known cause for CCSK, doctors believe that kidney cells may form improperly during development. This may form a tumor or create conditions for a tumor to grow later.

Who gets clear cell sarcoma of the kidney?

Representing only about 4% of pediatric renal tumors, clear cell sarcoma of the kidney affects boys and girls equally. Patients with CCSK are usually diagnosed between the ages of 2 and 4.

What are the signs and symptoms of clear cell sarcoma of the kidney in kids?

Many symptoms of CCSK, such as fever, constipation and vomiting can also be attributed to other common illnesses. However, the more obvious symptoms of CCSK include:

  • An abdominal mass
  • Hematuria (blood in the urine)
  • Abdominal swelling
  • Abdominal pain
  • Back pain

If you do feel a mass in your child's abdomen, avoid applying any pressure to it. In the rare case it is a tumor, pressing on it may cause it to rupture.

How do we diagnose clear cell sarcoma of the kidney?

If your child has signs or symptoms of CCSK, your doctor will start with a physical examination of your child. Next, they will conduct blood and urine tests to determine how well the kidneys are functioning.

If the results of these tests point to a possible tumor, your doctor and a team of radiologists will perform imaging tests. Instruments such as an ultrasound, a computed tomography (CT) scan or magnetic resonance imaging (MRI) may be used to take images of your child’s abdomen. Your doctor will diagnose CCSK if the images show a tumor and it is consistent with clear cell sarcoma of the kidney.

Why choose us to test for clear cell sarcoma of the kidney?

Our Urologic Tumor Program is led by a team of experts in diagnosing and treating pediatric clear cell sarcoma of the kidney. We take extra precautions when taking ultrasounds, CT scans and MRIs to make sure your child is safe and comfortable.

The knowledge of our radiology team combined with our modern imaging technology translates to faster, more accurate results. This means we can complete all the tests and scans in one day to help reduce the stress for the parents and children.

Our team of child life specialists also helps to ease kids' anxiety and make them feel comfortable during the scans.

How is clear cell sarcoma of the kidney treated?

To treat CCSK, surgeons typically remove the tumor and lymph nodes around the kidney. Sometimes they need to remove the kidney completely, which is called a nephrectomy. If the tumor has spread outside the kidney, your child may need additional surgery, medication or radiation therapy.

Why choose us for treatment of clear cell sarcoma of the kidney?

At Children's Colorado, we have extensive experience treating and managing clear cell sarcoma of the kidney in children. Our medical experts are also active in urologic cancer clinical trials and research and strive to find more effective and innovative ways to treat CCSK. We are also fortunate to have one of the only pediatric urologists in the world who is also fellowship-trained in urologic oncology, Nicholas Cost, MD. In addition to diagnosing and treating childhood CCSK, Dr. Cost leads research teams specializing in kidney tumors and has written numerous articles about the subject.

When it comes to surgical treatment for pediatric kidney tumors, we are also able to offer minimally invasive surgery (laparoscopic and robotic-assisted surgery) in suitable cases. Minimally invasive surgery provides the highest precision as well as shorter recovery times, which helps children get back to their normal lives faster.

Helpful CCSK resources

We recommend the following resource for research and information about CCSK:

  • 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