What comprises appropriate therapy for children adolescents with rhabdomyosarcama arising in the abdominal wall? A report from the Intergroup Rhabdomyosarcoma Study group

Purpose: The aim of this study was to define clinical features and determin e the best therapy for patients with rhabdomyosarcoma (RMS) of the abdomina l wall. Methods: We examined the demographic, clinical features, therapy (especiall y surgical), and outcome of 34 patients. Patients received combination chem otherapy after complete surgical resection (group I, n=14; 41%); resection with microscopic residual followed by local irradiation (RT; group II, n = 8; 24%); partial resection or biopsy only plus RT with gross locoregional r esidual tumor (group III, n = 4; 12%); or biopsy only plus RT with metastat ic disease (group IV, n = 8; 24%). Patients with group I or group II tumors had undergone partial abdominal wall resection (ie, involved muscle only w ith preservation of peritoneum, n = 11) or complete abdominal wall resectio n (n = 7). Four additional patients had groin lesions. Results: Thirty-four children or adolescents with abdominal wall RMS (about 1% of all patients) were treated on Inter-group Rhabdomyosarcoma Study I ( IRS-I) through IRS-IV. Overall, adolescents comprised 14 of 34 eligible pat ients (41%), and 10 of 14 (71%) adolescents had alveolar or undifferentiate d tumors versus 8 of 20 (40%) younger children (P = .07), Failure-free surv ival (FFS) rate and survival rate at 5 years was 65%. Treatment outcome was poorer for patients with group III-IV tumors (P = .01), adolescents (P = . 09) and patients with alveolar or undifferentiated sarcomas (P = .12). Conclusion: Patients with localized tumors appear to fare better if they un dergo complete abdominal wall resection (long-term survival rate, 100%) ver sus partial resection (longterm survival rate, 62% [P = .12]). Copyright (C ) 1999 by W.B. Saunders Company.