PURPOSE: To correlate TNM stage with clinical group as a function of p
rimary site in children with rhabdomyosarcoma. MATERIALS AND METHODS:
In 547 children with nonmetastatic rhabdomyosarcoma, the disease was s
taged prospectively by using a TNM system and grouped according to res
ectability of the primary tumor. These factors were reviewed for each
primary site. RESULTS: Primary tumor T status (T1, noninvasive; T2, in
vasive) was predictive of clinical group; 99 (36%) of 278 T1 tumors an
d 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 (7
4%) 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 treat
ment, whether irradiation or resection, or both, should be determined
according to TNM stage, age of child, and site of primary tumor. All c
hildren should receive chemotherapy. The goal of modern therapy is lon
g-term survival, with maintenance of organ function and a good quality
of life.