Rhabdomyosarcoma involving the genitourinary organs, retroperitoneum, and pelvis

We report on 17 patients with rhabdomyosarcoma of the genitourinary organs, retroperitoneal, pelvic, or perineal regions. Treatment was refused by three patients with either bladder or prostatic lesions. The remaining patients were treated with combined modality therapy. Five of 6 patients with bladder lesions are free of tumor 24 to 108 months (mean 51) after diagnosis, while one patient died 4 months postoperatively; two patients with paratesticular lesions are free of disease 3 years and 10 months following diagnosis, respectively. Of two patients with perineal lesions, one patient died, probably due to metastases 21 months after diagnosis, while another patient with local tumor recurrence was treated by reexcision with follow-up for 12 months without evidence of tumor recurrence. The worst results were obtained with the four patients with retroperitoneal pelvic lesions; one patient died 3 months after gross resection of tumor, and the other three patients deteriorated in the 4 months after diagnosis. Overall, 7 of 8 operated patients with bladder or paratesticular lesions are free of disease with follow-up for 10 to 108 months. The biological behavior of embryonal rhabdomyosarcoma, with the sex and age distribution and the management are discussed.