Advances in the treatment of rhabdomyosarcoma

Rhabdomyosarcoma is the most common soft tissue sarcoma in children under 15 years of age. Several decades ago it was associated with an 80% mortality rate. Recent advances with combined modality therapy have improved the 5-year survival rate to almost 70%. The focus for the improvement in treatment regimens is now shifting to concerns regarding the preservation of body function and the treatment of far advanced disease. Regardless of age, histology, stage, or site of primary tumor, there has been no group where chemotherapy has not been proven beneficial. The current recommendations used by the Intergroup Rhabdomyosarcoma Study, with regard to chemotherapy, are summarized. Radiation therapy adjunctive to surgery has a useful place in the treatment of rhabdomyosarcoma, but prophylactic nodal irradiation is not recommended. Involved nodes should, however, be treated. Since the potentially adverse side effects of chemotherapy and radiation are accentuated when combined, radiation therapy is no longer recommended in patients with group I rhabdomyosarcoma. Prognostic factors and future considerations from the operative standpoint are presented.