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Effect of Duration of Treatment on Treatment Outcome for Patients With Clear-Cell Sarcoma of the Kidney: A Report From the National Wilms' Tumor Study Group

Nita L. Seibel, Sierra Li, Norman E. Breslow, J. Bruce Beckwith, Daniel M. Green, Gerald M. Haase, Michael L. Ritchey, Patrick R.M. Thomas, Paul E. Grundy, Jerry Z. Finklestein, Tae Kim, Stephen J. Shochat, Panayotis P. Kelalis, Giulio J. D'Angio

From the Department of Hematology/Oncology, Children's National Medical Center; The George Washington University School of Medicine, Washington, DC; Department of Biostatistics, University of Washington; Fred Hutchinson Cancer Research Center, Seattle, WA; Department of Pathology, Loma Linda University, Loma Linda; Harbor-University of California Los Angeles Medical Center, Torrance; Jonathan Jacques Children's Cancer Center, Miller Children's Hospital, Long Beach, CA; Department of Pediatrics, Roswell Park Cancer Institute; School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York, Buffalo, NY; Department of Pediatric Surgery, Denver Children's Hospital, Denver, CO; Department of Pediatric Surgery, University of Texas at Houston Health Science Center, Houston, TX; Department of Radiation Oncology, Temple University School of Medicine; Department of Radiation Oncology, Hospital of the University of Pennsylvania, Philadelphia, PA; Departments of Pediatrics and Oncology, Cross Cancer Institute; University of Alberta, Edmonton, Alberta, Canada; Abbott Northwestern Hospital, Minneapolis, MN; and Department of Surgery, St Jude Children's Research Hospital, Memphis, TN.

Address reprint requests to Nita L. Seibel, MD, Department of Hematology/Oncology, Children's National Medical Center, 111 Michigan Ave, NW, Washington, DC 20010; e-mail: nseibel{at}cnmc.org

PURPOSE: To evaluate the effect of conventional and standard (ST) versus pulse-intensive (PI) chemotherapy and short-duration versus long-duration chemotherapy on relapse-free survival (RFS) and overall survival rates of patients with clear-cell sarcoma of the kidney (CCSK) entered onto the National Wilms' Tumor Study (NWTS)-4.

PATIENTS AND METHODS: The 5-year and 8-year RFS rates were determined for patients with CCSK treated on the NWTS-4. After August 6, 1986, 40 previously untreated children younger than 16 years with CCSK were randomly assigned, after the completion of 6 months of chemotherapy, to discontinue (short) or continue 9 additional months (long) of treatment with chemotherapy regimens that included vincristine and either divided-dose (ST) courses (5 days) or single-dose (PI) treatment with dactinomycin and divided-dose (ST) courses (3 days) or single-dose (PI) treatment with doxorubicin.

RESULTS: For patients with CCSK, the 5- and 8-year RFS rates were 65.2% and 60.6%, respectively, for patients randomly assigned to the short chemotherapy and 87.8% (both 5- and 8-year RFS) for patients randomly assigned to the long chemotherapy (P = .08). The overall survival rates for patients at 5 and 8 years were 95.5% and 85.9%, respectively, for the short chemotherapy and 87.5% (both 5- and 8-year overall survival) for the long chemotherapy (P = .99). In NWTS-4, the overall survival rates for patients with CCSK improved from NWTS-3 (83% v 66.9% at 8 years, respectively; P < .01).

CONCLUSION: CCSK patients exhibit an improved RFS from a longer course of therapy when using vincristine, doxorubicin, and dactinomycin, but their long-term survival is unchanged compared with patients receiving 6 months of therapy. The overall survival rates for patients with CCSK have improved from NWTS-3.

Deceased.

Supported in part by United States Public Health Service grant no. CA-42326.

Authors' disclosures of potential conflicts of interest are found at the end of this article.

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