TREATMENT OF CHILDREN WITH STAGE-II TO STAGE-IV ANAPLASTIC WILMS-TUMOR - A REPORT FROM THE NATIONAL-WILMS-TUMOR-STUDY-GROUP

Purpose: To evaluate the effect of the combination of vincristine, dac tinomycin, and doxorubicin with (regimen J) or without (regimen DD-RT) cyclophosphamide on the relapse-free survival of children with stages II to IV Wilms' tumor and focal or diffuse anaplasia. Patients and Me thods: We reviewed the clinical course of all randomized patients from National Wilms' Tumor Study (NWTS)-3 and NWTS-4 with stages II to IV anaplastic Wilms' tumor, and determined the 4-year relapse-free surviv al rate separately for those with focal or diffuse anaplasia. Anaplasi a was evaluated using newly developed topographic definitions for foca l and diffuse anaplasia. Results: The 4-year relapse-free survival rat e for five children with focal anaplasia who received regimen DD-RT wa s 80.0%, compared with 100.0% for eight children who received regimen J (P = .68). The 4-year relapse-free survival rate for 29 children wit h diffuse anaplasia treated with regimen DD-RT was 27.2%, compared wit h 54.8% for 30 children treated with regimen J (P = .02). Conclusion: We conclude that children with focal anaplasia have an excellent progn osis when treated with vincristine, doxorubicin, and dactinomycin. The addition of cyclophosphamide to the three-drug treatment regimen impr oved the 4-year relapse-free survival rate of children with stage II t o IV diffuse anaplasia. This result suggests that further intensificat ion of the treatment regimen for children with diffuse anaplasia may r esult in an additional improvement in prognosis. (C) 1994 by American Society of Clinical Oncology.