PURPOSE: 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). PATIENTS AND 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). CONCLUSION: 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.
RCT Entities:
PURPOSE: 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). PATIENTS AND 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). CONCLUSION: 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: Saskia L Gooskens; Norbert Graf; Rhoikos Furtwängler; Filippo Spreafico; Christophe Bergeron; Gema L Ramírez-Villar; Jan Godzinski; Christian Rübe; Geert O Janssens; Gordan M Vujanic; Ivo Leuschner; Aurore Coulomb-L'Hermine; Anne M Smets; Beatriz de Camargo; Sara Stoneham; Harm van Tinteren; Kathy Pritchard-Jones; Marry M van den Heuvel-Eibrink Journal: Nat Rev Urol Date: 2018-02-27 Impact factor: 14.432
Authors: Nita L Seibel; Yueh-Yun Chi; Elizabeth J Perlman; Jing Tian; Junfeng Sun; James R Anderson; Michael L Ritchey; Patrick R Thomas; James Miser; John A Kalapurakal; Paul E Grundy; Daniel M Green Journal: Pediatr Blood Cancer Date: 2018-09-25 Impact factor: 3.167
Authors: S L Gooskens; R Furtwängler; F Spreafico; H van Tinteren; J de Kraker; G M Vujanic; I Leuschner; A Coulomb-L'Herminé; J Godzinski; G Schleiermacher; S Stoneham; C Bergeron; K Pritchard-Jones; N Graf; M M van den Heuvel-Eibrink Journal: Br J Cancer Date: 2014-06-17 Impact factor: 7.640