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Clear Cell Sarcoma of the Kidney

Clear cell sarcoma of the kidney (CCSK) in children is a rare kidney tumor representing approximately 3% of all kidney tumors. This tumor is considered more aggressive than Wilms tumor and has a tendency to spread to the bones and brain. With the current treatment regimens, the prognosis for patients with CCSK is very good.

Evaluation and Treatment

Children with clear cell sarcoma of the kidney usually present with an abdominal mass. Sometimes they can present with blood in the urine, abdominal pain or increased blood pressure. At Texas Children’s Cancer and Hematology Center, a multidisciplinary team of physician scientists are involved in the diagnosis and treatment of children with CCSK. We use the latest molecular diagnostic methods to accurately diagnose children with CCSK.  We treat them with combination of surgery, radiation therapy and chemotherapy.

Pediatric clear cell sarcoma of the kidney registry

At Texas Children’s Cancer and Hematology Center, we are interested in understanding the biology of CCSK with the goal of offering more successful therapies for pediatric patients. Our researchers uncovered a genetic mutation associated with CCSK that was published in Nature Communications. We have now developed an innovative molecular test to accurately diagnose CCSK in children.

We have also established the pediatric clear cell sarcoma of the kidney registry to collect information on children with CCSK. Email us at to get information on how to enroll in the registry.