Factors that influence treatment decisions in childhood rhabdomyosarcoma

PURPOSE: To correlate TNM stage with clinical group as a function of primary site in children with rhabdomyosarcoma. MATERIALS AND METHODS: In 547 children with nonmetastatic rhabdomyosarcoma, the disease was staged prospectively by using a TNM system and grouped according to resectability of the primary tumor. These factors were reviewed for each primary site. RESULTS: Primary tumor T status (T1, noninvasive; T2, invasive) was predictive of clinical group; 99 (36%) of 278 T1 tumors and 238 (88%) of 269 T2 tumors were nonresectable (clinical group III) (P < .0001). Tumor size (less than or equal to 5 cm or >5 cm) was also predictive of clinical group; 111 (46%) of 240 small tumors and 226 (74%) of 307 large tumors were nonresectable (P < .0001). Regional lymph nodes (N1) were found in 62 (24%) of 253 T2 tumors but in only 21 (8%) of 277 T1 tumors and in 67 (23%) of 294 large tumors but only 16 (7%) of 236 small tumors. Site of primary tumor was predictive of outcome and correlated with local treatment received. CONCLUSION: Local treatment, whether irradiation or resection, or both, should be determined according to TNM stage, age of child, and site of primary tumor. All children should receive chemotherapy. The goal of modern therapy is long-term survival, with maintenance of organ function and a good quality of life.