Treatment and outcomes of clear cell sarcoma of the kidney: A report from the Children's Oncology Group studies AREN0321 and AREN03B2


Daniel J. Benedetti, Division of Hematology/Oncology, Department of Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Lindsay A. Renfro, Division of Biostatistics, University of Southern California, Los Angeles, California, USA.
Ian Tfirn, Children's Oncology Group, Monrovia, California, USA.
Najat C. Daw, Division of Pediatrics, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
John A. Kalapurakal, Department of Radiation Oncology, Northwestern University, Chicago, Illinois, USA.
Peter F. Ehrlich, Section of Pediatric Surgery, University of Michigan, Ann Arbor, Michigan, USA.
Geetika Khanna, Department of Radiology and Imaging Sciences, Emory University, Children's Healthcare of Atlanta, Atlanta, Georgia, USA.
Elizabeth Perlman, Department of Pathology and Laboratory Medicine, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Anne Warwick, Department of Pediatrics, F. Edward Hébert School of Medicine, Uniformed Services University, Bethesda, Maryland, USA.
Kenneth W. Gow, Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, University of Washington, Seattle, Washington, USA.
Arnold C. Paulino, Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Nita L. Seibel, Division of Cancer Treatment and Diagnosis, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.
Paul Grundy, Division of Immunology, Hematology, Oncology, Palliative Care, and Environmental Interactions, University of Alberta, Edmonton, Alberta, Canada.
Conrad V. Fernandez, Division of Pediatric Hematology/Oncology, IWK Health Centre, Dalhousie University, Halifax, Nova Scotia, Canada.
James I. Geller, Division of Oncology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio, USA.
Elizabeth A. Mullen, Department of Pediatric Hematology/Oncology, Dana-Farber Cancer Institute/Boston Children's Hospital, Boston, Massachusetts, USA.
Jeffrey S. Dome, Division of Oncology and Department of Pediatrics, Children's National Hospital and the George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA.

Document Type

Journal Article

Publication Date







brain metastases; carboplatin; clear cell sarcoma of the kidney (CCSK); radiotherapy


BACKGROUND: On the fifth National Wilms Tumor Study, treatment for clear cell sarcoma of the kidney (CCSK) included combined vincristine, doxorubicin, cyclophosphamide, and etoposide (regimen I) plus radiation therapy (RT), yielding 5-year event-free survival (EFS) rates of 100%, 88%, 73%, and 29% for patients who had with stage I, II, III, and IV disease, respectively. In the Children's Oncology Group study AREN0321 of risk-adapted therapy, RT was omitted for stage I disease if lymph nodes were sampled, and carboplatin was added for stage IV disease (regimen UH-1). Patients who had stage II/III disease received regimen I with RT. METHODS: Four-year EFS was analyzed for patients enrolled on AREN0321 and on those enrolled on AREN03B2 who received AREN0321 stage-appropriate chemotherapy. RESULTS: Eighty-two patients with CCSK enrolled on AREN0321, 50 enrolled on AREN03B2 only. The 4-year EFS rate was 82.7% (95% confidence interval [CI], 74.8%-91.4%) for AREN0321 and 89.6% (95% CI, 81.3%-98.7%) for AREN03B2 only (p = .28). When combining studies, the 4-year EFS rates for patients who had stage I (n = 10), II (n = 47), III (n = 65), and IV (n = 10) disease were 90% (95% CI, 73.2%-100.0%), 93.4% (95% CI, 86.4%-100.0%), 82.8% (95% CI, 74.1%-92.6%), and 58.3% (95% CI, 34%-100.0%), respectively. There were no local recurrences among seven patients with stage I disease who were treated without RT. One stage I recurrence occurred in the brain, which was the most common site of relapse overall. Among patients with local stage III tumors, neither initial procedure type, margin status, nor lymph node involvement were prognostic. CONCLUSIONS: Patients with stage I CCSK had excellent outcomes without local recurrences when treated without RT. Patients with stage IV disease appeared to benefit from a carboplatin-containing regimen, although their outcomes remained unsatisfactory. Further research is needed to improve outcomes for patients with advanced-stage disease ( identifiers NCT00335556 and NCT00898365).