Results of the Intergroup Rhabdomyosarcoma Study Group D9602 protocol, using vincristine and dactinomycin with or without cyclophosphamide and radiation therapy, for newly diagnosed patients with low-risk embryonal rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group
Department of Radiation Oncology; Quality Assurance Review Center
Adolescent; Adult; Antineoplastic Combined Chemotherapy Protocols; Child; Child, Preschool; Cyclophosphamide; Dactinomycin; Disease-Free Survival; Female; Humans; Kaplan-Meier Estimate; Lymphatic Metastasis; Male; Neoplasm Recurrence, Local; Neoplasm Staging; Proportional Hazards Models; Radiation Dosage; Radiotherapy, Adjuvant; Rhabdomyosarcoma, Embryonal; Risk Assessment; Risk Factors; Survival Rate; Time Factors; Treatment Outcome; United States; Vincristine; Young Adult
Neoplasms | Oncology
PURPOSE: Patients with localized, grossly resected, or gross residual (orbital only) embryonal rhabdomyosarcoma (ERMS) had 5-year failure-free survival (FFS) rates of 83% and overall survival rates of 95% on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III/IV. IRSG D9602 protocol (1997 to 2004) objectives were to decrease toxicity in similar patients by reducing radiotherapy (RT) doses and eliminating cyclophosphamide for the lowest-risk patients.
PATIENTS AND METHODS: Subgroup A patients (lowest risk, with ERMS, stage 1 group I/IIA, stage 1 group III orbit, stage 2 group I) received vincristine plus dactinomycin (VA). Subgroup B patients (ERMS, stage 1 group IIB/C, stage I group III nonorbit, stage 2 group II, stage 3 group I/II) received VA plus cyclophosphamide. Patients in group II/III received RT. Compared with IRS-IV, doses were reduced from 41.4 to 36 Gy for stage 1 group IIA patients and from 50 or 59 to 45 Gy for group III orbit patients.
RESULTS: Estimated 5-year FFS rates were 89% (95% CI, 84% to 92%) for subgroup A patients (n = 264) and 85% (95% CI, 74%, 91%) for subgroup B patients (n = 78); median follow-up: 5.1 years. Estimated 5-year FFS rates were 81% (95% CI, 68% to 90%) for patients with stage 1 group IIA tumors (n = 62) and 86% (95% CI, 76% to 92%) for patients with group III orbit tumors (n = 77).
CONCLUSION: Five-year FFS and OS rates were similar to those observed in comparable IRS-III patients, including patients receiving reduced RT doses, but were lower than in comparable IRS-IV patients receiving VA plus cyclophosphamide. Five-year FFS rates were similar among subgroups A and B patients.
DOI of Published Version
J Clin Oncol. 2011 Apr 1;29(10):1312-8. doi: 10.1200/JCO.2010.30.4469. Link to article on publisher's site
Journal of clinical oncology : official journal of the American Society of Clinical Oncology
Raney RB, Walterhouse DO, Meza JL, Andrassy RJ, Breneman JC, Crist WM, Maurer HM, Meyer WH, Parham DM, Anderson JR. (2011). Results of the Intergroup Rhabdomyosarcoma Study Group D9602 protocol, using vincristine and dactinomycin with or without cyclophosphamide and radiation therapy, for newly diagnosed patients with low-risk embryonal rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group. Radiation Oncology Publications. https://doi.org/10.1200/JCO.2010.30.4469. Retrieved from https://escholarship.umassmed.edu/radiationoncology_pubs/55