title: Treatment of children with clear-cell sarcoma of the kidney: a report from the National Wilms' Tumor Study Group.
abstract: OBJECTIVE
  To evaluate the effect of the sequential addition of doxorubicin (DOX) and cyclophosphamide (CTX) to the combination of vincristine (VCR) and dactinomycin (AMD) on the relapse-free survival of children with clear-cell sarcoma of the kidney (CCSK).
  
  
  METHODS
  We determined the 6-year relapse-free survival rate for patients with CCSK treated on National Wilms' Tumor Study (NWTS)-1, NWTS-2, or NWTS-3 with the combination of VCR and AMD, with or without DOX, and for patients treated on NWTS-3 with the combination of VCR, AMD, and DOX with (regimen J) or without (regimen DD-RT) CTX.
  
  
  RESULTS
  The 6-year relapse-free survival rate for the eight children with CCSK treated with VCR, AMD, and radiation therapy was 25.0%, compared with 63.5% for the 58 children treated with VCR, AMD, DOX, and radiation therapy (P = .09). The 6-year relapse-free survival rate for children with CCSK treated on regimen DD-RT was 64.6%, compared with 58.2% for those treated on regimen J (P = .79).
  
  
  CONCLUSIONS
  We conclude that the addition of DOX to the combination of VCR plus AMD appeared to improve the 6-year relapse-free survival rate of children with CCSK. The addition of CTX in the dose and schedule used in NWTS-3 did not improve the 6-year relapse-free survival rate of children with CCSK. Because 30% of relapses occurred more than 2 years after diagnosis, prolonged follow-up evaluation of patients with CCSK is necessary.
authors.affiliation: Department of Pediatrics, Roswell Park Cancer Institute, Buffalo, NY 14263.
authors.forename: D M
authors.initials: DM
authors.lastname: Green
authors.forename: N E
authors.initials: NE
authors.lastname: Breslow
authors.forename: J B
authors.initials: JB
authors.lastname: Beckwith
authors.forename: J
authors.initials: J
authors.lastname: Moksness
authors.forename: J Z
authors.initials: JZ
authors.lastname: Finklestein
authors.forename: G J
authors.initials: GJ
authors.lastname: D'Angio
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000188:drug therapy* / Q000532:radiotherapy; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000188:drug therapy* / Q000532:radiotherapy; D016016:Proportional Hazards Models; D014481:United States; D014750:Vincristine / Q000008:administration & dosage; D009396:Wilms Tumor
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide