[Rhabdomyosarcoma: therapeutic results and prospectives]

The outlook for children with rhabdomyosarcoma has change significantly in these last years. With an adeguate combined modality therapy more than 50% of these children may be cured. The results of the IRS-I indicate that the 3 year relapse-free survival rates are 85% for patients in group I 70% for those in group II, 45% for those in group III and 15% for those in group IV. In addition to the clinical group other significant prognostic factors are histologic cell type (alveolar, unfavorable) and primary site (disease in extremities and in retroperitoneal area, unfavorable). The chemiotherapy must be used in all patients for 12-24 months. The effective drugs are VCR, ACT-D, CTX, ADR combined in different schedules. It has been demonstrated that the effective doses of radiotherapy range from 4000 to 5000 rad and that radiotherapy may be omitted in patients in group I. Now a less aggressive surgical procedures may be employed, and patients with primary tumor in the orbit or in the pelvic organs may be cured saving the eye, or the bladder, the vagina and the uterus.