Surgical management of gynecologic rhabdomyosarcoma.

The multidisciplinary treatment strategy for rhabdomyosarcoma (RMS) of the female gynecologic system is evidence of the progress made in the management of many childhood cancers. Previously, initial radical surgery (eg, complete vaginectomy, hysterectomy, and pelvic exenteration) and less effective single-agent chemotherapeutic agents were used with suboptimal results. Over the past 30 years, there has been an increase in overall survival with gynecologic RMS and a significant decrease in the hysterectomy and vaginectomy rates. The usual recommended treatment strategy for gynecologic RMS is initial biopsy to establish the diagnosis, followed by neoadjuvant chemotherapy. The specific chemotherapy regimen is selected based on the risk stratification of the particular patient, and this will often allow a more conservative surgical approach to be used, with the goal of complete tumor resection. Radiation therapy is considered before definitive surgical resection with minimal response to chemotherapy or after definitive surgical resection with positive margins. Recurrent or persistent RMS, including RMS of the female gynecologic system, represents a very aggressive disease with poor outcome. In these cases, aggressive management, including experimental chemotherapy agents and organ-sacrificing surgery, is often indicated to afford any chance of long-term survival. Continued enrollment of a high proportion of these patients into ongoing prospective clinical trials should allow the outcomes to continue to improve.