Genitourinary rhabdomyosarcoma

Rhabdomyosarcoma is the most common soft tissue malignant neoplasm involving the pelvis of children. Debate still exists over whether the best treatment is pelvic exenteration, radiation and chemotherapy or chemotherapy as the cornerstone to treatment, with diminished needs for extensive surgery and prolonged radiotherapy. Contrariwise, there is little debate over the combined treatment modality for paratesticular rhabdomyosarcoma. We have evaluated and treated 19 children with rhabdomyosarcoma, including 17 with pelvic rhabdomyosarcoma and 2 with paratesticular rhabdomyosarcoma. This restrospective study was done to evaluate treatment regimens for patients with genitourinary rhabdomyosarcoma. For patients with pelvic rhabdomyosarcoma the study was divided into 2 series. In an early series 6 of 7 children had a pelvic exenteration with or without chemotherapy. Of these 7 children 3 are well 15 to 27 years following diagnosis. In a later series of children, when chemotherapy was used more commonly, none underwent pelvic exenteration. Of these 10 patients 7 had chemotherapy or chemotherapy and biopsy only. Only 1 child, who presented with stage IV disease, died in this series. Another child with stage III disease had progressive disease despite chemotherapy and subsequent cystoprostatectomy. Thus, of 9 remaining patients 8 are well from 1 to 8 years. It appears that pelvic rhabdomyosarcoma can be treated effectively with chemotherapy, and limited surgery and radiation. Fortunately, pelvic exenteration can now be limited to a select few.