Bladder/prostate rhabdomyosarcoma: results of the multi-institutional trials of the Intergroup Rhabdomyosarcoma Study

Rhabdomyosarcomas (RMS) occurring in pelvic sites are almost entirely of the embryonal histologic subtype and are relatively responsive to chemotherapeutic agents. When treated by radical surgery, pelvic irradiation, and intensive chemotherapy for 2 years, survival rates of higher than 85% have been achieved. However, the effects of such therapeutic approaches have resulted in major changes in the quality of life of survivors, because of the loss of pelvic organs, primarily the bladder. Current efforts are directed towards achieving long range survival with procedures that are less extensive than pelvic exenteration or total cystectomy and by regimens that eliminate or reduce the intensity of radiotherapy. Until the past 5 years, these efforts have been relatively unsuccessful in both North America and Europe. However, the most recent studies of the IRS and European cooperative groups suggest that a majority of these patients can survive with intact pelvic organs when treated with currently available chemotherapeutic agents in conjunction with radiotherapy and limited surgery.