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

• Published in: Journal of clinical oncology Vol. 29; no. 10; pp. 1312 - 1318
• Main Authors: BEVERLY RANEY, R, WALTERHOUSE, David O, MEZA, Jane L, ANDRASSY, Richard J, BRENEMAN, John C, CRIST, William M, MAURER, Harold M, MEYER, William H, PARHAM, David M, ANDERSON, James R
• Format: Journal Article
• Language: English
• Published: Alexandria, VA American Society of Clinical Oncology 01.04.2011
• Subjects: Adolescent; Adult; Antineoplastic Combined Chemotherapy Protocols - adverse effects; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Child; Child, Preschool; Cyclophosphamide - administration & dosage; Dactinomycin - administration & dosage; Disease-Free Survival; Diseases of the osteoarticular system; Female; Humans; Kaplan-Meier Estimate; Lymphatic Metastasis; Male; Medical sciences; Neoplasm Recurrence, Local; Neoplasm Staging; Original Reports; Proportional Hazards Models; Radiation Dosage; Radiotherapy, Adjuvant; Rhabdomyosarcoma, Embryonal - drug therapy; Rhabdomyosarcoma, Embryonal - mortality; Rhabdomyosarcoma, Embryonal - radiotherapy; Rhabdomyosarcoma, Embryonal - secondary; Risk Assessment; Risk Factors; Survival Rate; Time Factors; Treatment Outcome; Tumors; Tumors of striated muscle and skeleton; United States; Vincristine - administration & dosage; Young Adult; United States; Antineoplastic agent; Human; Embryonal rhabdomyosarcoma; Soft tissue sarcoma; Early stage; Malignant tumor; Dactinomycin; Radiotherapy; Vincristine; Low risk; Alkylating agent; Alkaloid; Oxazaphosphinane derivatives; Striated muscle disease; Cyclophosphamide; Cancerology; Treatment; Aminoglycoside; Nitrogen mustard; Protein synthesis inhibitor; Antimitotic; Child; Cancer
• ISSN: 0732-183X; 1527-7755
• DOI: 10.1200/JCO.2010.30.4469

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. 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. 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). 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.