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    Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Clear Cell Sarcoma of the Kidney

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    Standard Treatment Options for Clear Cell Sarcoma of the Kidney

    Because of the relative rarity of this tumor, all patients with clear cell sarcoma of the kidney should be considered for entry into a clinical trial. Treatment planning by a multidisciplinary team of cancer specialists (pediatric surgeon or pediatric urologist, pediatric radiation oncologist, and pediatric oncologist) with experience treating renal tumors is required to determine and implement optimum 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 undergo postoperative radiation to the tumor bed and receive doxorubicin as part of their chemotherapy regimen.

    The standard treatment option for clear cell sarcoma of the kidney is the following:

    1. Surgery, chemotherapy, and radiation therapy.

    Surgery, chemotherapy, and radiation therapy

    Evidence (surgery, chemotherapy, and radiation therapy):

    1. In the NWTS-3 trial, the addition of doxorubicin to the combination of vincristine, dactinomycin, and radiation therapy resulted in an improvement in disease-free survival for patients with clear cell sarcoma of the kidney.[1]
    2. The NWTS-4 trial used regimen DD-4A, which consisted of vincristine, dactinomycin, and doxorubicin for 15 months and radiation therapy.[6]
      • NWTS-4 reported that patients who were treated with vincristine, doxorubicin, and dactinomycin for 15 months had an improved relapse-free survival compared with patients who were treated for 6 months (88% vs. 61% at 8 years).
    3. In the NWTS-5 (COG-Q9401/NCT00002611) trial, children with stages I to IV clear cell sarcoma of the kidney were treated with a new chemotherapeutic regimen combining vincristine, doxorubicin, cyclophosphamide, and etoposide in an attempt to further improve the survival of these high-risk groups. All patients received radiation therapy to the tumor bed.[7]
      • With this treatment, the 5-year EFS was approximately 79%, and the OS was approximately 89%.
      • Stage I patients had 5-year EFS and OS rates of 100%.
      • Stage II patients had a 5-year EFS of approximately 87% and a 5-year OS of approximately 97%.
      • Stage III patients had a 5-year EFS of approximately 74% and a 5-year OS of approximately 87%.
      • Stage IV patients had a 5-year EFS of approximately 36% and a 5-year OS of 45%.
    4. A review of stage I clear cell sarcoma of the kidney treated on the NWTS-1 through NWTS-5 trials showed an excellent 100% OS with a wide variety of chemotherapy and radiation therapy regimens.[9]
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