The role of surgery in the management of pediatric pelvic rhabdomyosarcoma

Abstract

Purpose: We assessed the role of surgery, particularly exenteration, in the treatment of children with lower urinary tract and pelvic rhabdomyosarcoma.

Materials and methods: 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.

Results: 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).

Conclusions: Exenterative surgery is frequently required to achieve a durable complete response.