Bladder and/or Prostate Rhabdomyosarcoma

Abstract

Rhabdomyosarcoma (RMS) is a soft tissue malignant tumor of mesenchymal origin. It is the third most common solid tumor in children accounting for 10–15 % of all pediatric solid tumors [1]. The incidence of RMS is estimated at 4.5 per 1 million, with over 50 % of cases diagnosed in the first decade of life [1, 2]. RMS exhibits a bimodal distribution for age at presentation with a peak between 2 and 6 years, and then again between 10 and 18 years of age [3]. Approximately 15–20 % of RMS tumors arise in the genitourinary (GU) system primarily in the prostate, bladder, and para-testicular area [4]. Vaginal, cervical, uterine, primary retroperitoneal, and renal RMS are relatively unusual sites. The treatment of GU-RMS has drastically evolved over the years due to effective multidisciplinary, multimodal, and risk adapted therapies developed through clinical trials. Prognosis has significantly improved as a result of all these measures, currently with an expected overall 5-year survival >70 % [1]. Accordingly, the goals in treating GU-RMS have shifted towards minimizing morbidity and reducing therapy. Surgery has shifted from pelvic exenteration to organ preservation. Surgery is directed by stage, risk stratification, and response to chemotherapy/radiation therapy. Current relevant surgical procedures for the treatment of RMS include performing a biopsy to establish a diagnosis, and subsequent partial cystectomy, cystectomy ± prostatectomy (cystoprostatectomy), and pelvic lymphadenectomy. For para-testicular RMS the reader is recommended to review the chapter on testicular tumors and associated procedures such as orchiectomy and retroperitoneal lymph node dissection.