title: 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.
abstract: OBJECTIVE
  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.
  
  
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Hematology/Oncology, Children's National Medical Center, 111 Michigan Avenue NW, Washington, DC 20010, USA. nseibel@cnmc.org
authors.forename: Nita L
authors.initials: NL
authors.lastname: Seibel
authors.forename: Sierra
authors.initials: S
authors.lastname: Li
authors.forename: Norman E
authors.initials: NE
authors.lastname: Breslow
authors.forename: J Bruce
authors.initials: JB
authors.lastname: Beckwith
authors.forename: Daniel M
authors.initials: DM
authors.lastname: Green
authors.forename: Gerald M
authors.initials: GM
authors.lastname: Haase
authors.forename: Michael L
authors.initials: ML
authors.lastname: Ritchey
authors.forename: Patrick R M
authors.initials: PR
authors.lastname: Thomas
authors.forename: Paul E
authors.initials: PE
authors.lastname: Grundy
authors.forename: Jerry Z
authors.initials: JZ
authors.lastname: Finklestein
authors.forename: Tae
authors.initials: T
authors.lastname: Kim
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Shochat
authors.forename: Panayotis P
authors.initials: PP
authors.lastname: Kelalis
authors.forename: Giulio J
authors.initials: GJ
authors.lastname: D'Angio
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003609:Dactinomycin / Q000008:administration & dosage; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007680:Kidney Neoplasms / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000517:prevention & control*; D009367:Neoplasm Staging; D018227:Sarcoma, Clear Cell / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin