Surgical lessons from the Intergroup Rhabdomyosarcoma Study

The Intergroup Rhabdomyosarcoma Study, initiated in 1972, has admitted more than 700 patients with childhood rhabdomyosarcoma in a 6-year period. Although it was designed primarily to answer specific questions about various aspects of radiotherapy and chemotherapy, some lessons have been learned concerning the surgical approach to this disease. Operative resection, which is not always feasible, is determined chiefly by the clinical stage and anatomic site. Thus far, data for some anatomic sites indicated that total gross resection at some point in the treatment schedule achieved better results than did incomplete resection. A possible exception to this occurred when the orbit is the primary site. The incidence of lymphatic metastases from lesions in some anatomic sites (e.g., paratesticular, pelvic genito-urinary, and extremity) was higher than was previously appreciated. This finding will influence decisions concerning surgical biopsy or dissection of regional lymph nodes for many of these lesions. Differences in histology, particularly those relating to the alveolar type, indicated the need for a more aggressive approach to selected lesions, but we need more data to confirm this determination. Lastly, patients who initially received radiotherapy or chemotherapy to pelvic sites have had striking benefits, and this finding should encourage the development of schedules that specify operative resection of these lesions later in the program rather than as the initial treatment.