Outcomes of patients with revised stage I clear cell sarcoma of kidney treated in National Wilms Tumor Studies 1-5.

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Manchanda V, Mohta A, Khurana N, Gupta CR, Neogi S. Outcome of clear cell sarcoma of the kidney (CCSK) treated on the National Wilms Tumor Study-5 (NWTS). Clear cell sarcoma of the kidney: a review of 351 cases from the National Wilms Tumor Study Group Pathology Center. Bone metastasizing renal tumor of childhood: morphological and clinical features, and differences from Wilms' tumor. Undifferentiated sarcoma of the kidney: a tumor of childhood with histopathologic and clinical characteristics distinct from Wilms' tumor. Histopathology and prognosis of Wilms tumors: results from the First National Wilms' Tumor Study. this treatment consists of a radical nephrectomy followed by radiotherapy and chemotherapy with cyclophosphamide, etoposide, vincristine, and doxorubicin for 24 weeks.īeckwith JB, Palmer NF. In the NWTS-5 protocol, patients with all stages of CCSK are treated with the same regimen used in patients who have Wilms tumor with diffuse anaplasia (excluding stage I) The 8-year relapse-free survival rate for localized clear cell sarcoma of the kidney stages I-III is 88%, but late relapses have been known to occur. The overall survival has improved for patients with clear cell sarcoma of the kidney from NWTS-3 to NWTS-4 (83% vs 66.9% at 8 y). NWTS-4 showed that patients treated with vincristine, doxorubicin, and dactinomycin for 15 months had an improved relapse-free survival rate compared with patients treated for 6 months (87.5% vs 60.6% at 8 y). In the third National Wilms Tumor Study (NWTS-3), the addition of doxorubicin to the combination of vincristine, dactinomycin, and radiation therapy resulted in an improvement in disease-free survival in patients with clear cell sarcoma of the kidney. The approach for treating clear cell sarcoma of the kidney (CCSK) is different from the approach for Wilms tumor because the overall survival of children with clear cell sarcoma of the kidney remains considerably lower than that of patients with favorable-histology Wilms tumor.

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