New approaches to the surgical management of rhabdomyosarcoma in childhood.

Recent trends in the management of pediatric solid-tumors have resulted in reducing the scope of surgery. In the therapy of RMS of the orbit, genitourinary tract, head & neck and trunk & extremity sites, regimens of primary chemotherapy/radiotherapy have replaced, reduced or delayed excisional surgery. In some sites, these approaches have been successful. In others, a return to a more active surgical approach may be indicated, as observed in two studies noted below. Results of a primary chemotherapy approach for vesical RMS have been disappointing, as noted in the Intergroup Rhabdomyosarcoma Study (IRS) (U.S.) in which the mortality is greater than 25% and rate of bladder salvage among survivors less than 45%. Partial cystectomy for RMS was justifiably condemned prior to the development of effective chemotherapy, but deserves reevaluation. Partial cystectomy with adjunctive chemotherapy was performed in 33 children, 21% of the total pts with vesical RMS in the IRS. In 27 of these patients, it was carried out as the initial operation. Six additional patients had tumors which responded to chemotherapy, and a secondary partial cystectomy was performed. The mortality among these patients has been the same as in the overall group of patients with primary bladder tumors. The retention of functional bladders, however, has been greater than 95% among survivors with only 1/26 bladders lost to contracture. All patients (4) in this group who had postoperative functional vesical problems received more irradiation than is currently recommended. In this study, the use of partial cystectomy has been confined to cases in which it would obviously be successful.(ABSTRACT TRUNCATED AT 250 WORDS)