Treatment results for patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III and IV, 1984-1997: A report from the Children's Oncology Group

• Published in: Pediatric blood & cancer 2010-10, Vol.55 (4), p.612-616
• Main Authors: Raney, R. Beverly, Anderson, James R., Brown, Kenneth L.B., Huh, Winston W., Maurer, Harold M., Meyer, William H., Parham, David M., Rodeberg, David A., Wolden, Suzanne L., Donaldson, Sarah S.
• Format: Article
• Language: English
• Subjects: Adolescent; Child; Child, Preschool; Female; Humans; Infant; Male; Neoplasm Staging; pediatric oncology; rhabdomyosarcoma; Rhabdomyosarcoma, Alveolar - mortality; Rhabdomyosarcoma, Alveolar - pathology; Rhabdomyosarcoma, Alveolar - therapy; soft tissue sarcoma; Treatment Failure
• ISSN: 1545-5009; 1545-5017
• DOI: 10.1002/pbc.22520

Purpose To assess local control, event‐free survival (EFS), and overall survival (OS) rates in 71 patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma (ALV RMS) and their relation to radiation therapy (RT) on IRSG Protocols III and IV, 1984–1997. Methods Chart review and standard statistical procedures. Patients and Tumors Patients were 1–18 years at diagnosis (median, 6 years). Primary tumor sites were extremity/trunk (N = 54), head/neck (N = 9), genitourinary tract (N = 7), and perineum (N = 1). Thirty patients received VA ± C with RT; 41 received VA ± C alone. RT was assigned, not randomized. Results Fifty‐four patients had Stage 1 (favorable site, any size) or Stage 2 (unfavorable site, ≤5 cm) tumors. Eight‐year EFS was 90%, with 100% local control for 17 patients given RT. Eight‐year EFS was 88%, with 92% local control for 37 patients without RT; P = 0.52 for EFS comparisons, 0.3 for local control comparisons. In 17 Stage 3 patients (unfavorable site, tumors >5 cm, N0), 8‐year EFS was 84% with 100% local control in 13 patients given RT; 8‐year EFS was only 25% and local control 50% in 4 patients without RT. Local recurrence was the most common site of first failure in non‐irradiated patients. Conclusion Patients with Stage 1–2 ALV RMS had slightly but statistically insignificantly improved local control, EFS, and OS rates when local RT was given. The need for local RT in Stage 1–2 patients deserves evaluation in a randomized study. Local control, EFS, and OS rates were significantly improved in Stage 3 patients receiving local RT. Pediatr Blood Cancer. 2010;55:612–616. © 2010 Wiley‐Liss, Inc.