The role of surgery in the management of pediatric pelvic rhabdomyosarcoma.

Abstract

We assessed the role of surgery, particularly exenteration, in the treatment of children with lower urinary tract and pelvic rhabdomyosarcoma. We treated 23 children with bladder and/or prostate (11), or pelvic retroperitoneal tumors (12). Initial management was tumor resection in 6 cases, anterior pelvic exenteration in 5 and biopsy only in 12, combined with chemotherapy in 23 and radiotherapy in 20. The bladder salvage rate for surviving patients with pelvic tumors was 92% versus 27% for those with prostate/bladder tumors. Estimated 5 and 10-year survival probability for patients with pelvic retroperitoneal tumors was 49 +/- 50% compared to 81 +/- 24% for those with bladder and/or prostate tumors (log rank test, p = 0.11). Exenterative surgery is frequently required to achieve a durable complete response.