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Treatment of Clear Cell Sarcoma of the Kidney
Source: Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 15, 2025 · Captured 2026-09-09.
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Because of the relative rarity of this tumor, all patients with clear cell sarcoma of the kidney should consider joining a clinical trial. Treatment planning by a multidisciplinary team of pediatric cancer specialists (surgeon or urologist, radiation oncologist, and oncologist) with experience treating renal tumors is required to determine and implement optimal treatment.
The approach for treating clear cell sarcoma of the kidney is different from the approach for treating Wilms tumor because the overall survival (OS) of children with clear cell sarcoma of the kidney remains lower than that for patients with FH Wilms tumor. All patients, except those who have lymph nodes sampled and are stage I, undergo postoperative radiation therapy to the tumor bed and receive doxorubicin as part of their chemotherapy regimen.
The standard treatment option for stage I (must have lymph nodes sampled) clear cell sarcoma of the kidney is the following:
Surgery and chemotherapy.[16]
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The standard treatment option for stages II through IV clear cell sarcoma of the kidney is the following:
Surgery, chemotherapy, and radiation therapy.[16]
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