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.