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Treatment of 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.

    Standard Treatment Options

    Recommended Related to Cancer

    Treatment Options for Gastrointestinal Stromal Tumors

    A link to a list of current clinical trials is included at the end of this section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you. Resectable Gastrointestinal Stromal Tumors Resectable gastrointestinal stromal tumors (GISTs) can be completely or almost completely removed by surgery. Treatment may include the following: Surgery to remove tumors that are 2 centimeters...

    Read the Treatment Options for Gastrointestinal Stromal Tumors article > >

    The approach for treating clear cell sarcoma of the kidney is different from Wilms tumor since the OS of children with clear cell sarcoma of the kidney remains lower than for patients with favorable histology Wilms tumor. In the National Wilms Tumor Study-3 (NWTS-3), 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] The National Wilms Tumor Study-4 (NWTS-4) showed that patients treated with vincristine, doxorubicin, and dactinomycin for 15 months had an improved relapse-free survival compared with patients treated for 6 months (88% vs. 61% at 8 years).[2] In the National Wilms Tumor Study-5 (COG-Q9401) 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. With this treatment, the 5-year EFS was approximately 79% and OS was approximately 89%. Stage I patients had 100% 5-year EFS and OS. Stage II patients had a 5-year EFS of approximately 87% and OS of approximately 97%. Stage III patients had an approximately 74% 5-year EFS and an approximately 87% 5-year OS. Stage IV patients had a 5-year EFS of approximately 36% and 5-year OS of 45%. Clear cell sarcoma of the kidney has been characterized by late relapses; however, in NWTS-5, most relapses occurred within 3 years. In NWTS-5, the most common site of recurrence was the brain,[3] which has been successfully treated with combination chemotherapy, surgery, and radiation therapy.[4][Level of evidence: 2A]

    • Regimen DD-4A (vincristine, dactinomycin, and doxorubicin) for 15 months and radiation therapy.[2]
    • Regimen I (vincristine, doxorubicin, cyclophosphamide, and etoposide) and radiation therapy.[3]
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