Extent of surgery in rhabdomyosarcoma of urogenital structures

After high inguinal semicastration in group-1 paratesticular rhabdomyosarcoma (RMS), the patient having undergone chemotherapy can be followed closely by CT scanning without retroperitoneal lymphadenectomy. In contrast, retroperitoneal RMS should be operated on as radically as possible after downstaging the tumor mass. In RMS of the female genitalia locally limited organ-preserving surgery is the method of choice. The prognosis is excellent with adjuvant chemotherapy. Only 20% of all bladder RMS arise from the bladder dome or the movable part of the bladder, where primary partial resection including a safety margin of 3 cm of healthy tissue is possible. The majority, however, arising from the submucous tissue of the bladder base, trigonal area and bladder neck, infiltrates the prostatic urethra and the surrounding pelvic fascia. Therefore differentiation between primary bladder or prostate RMS is often doubtful. The current strategy of downstaging by chemotherapy or chemo-radiotherapy followed either by limited organ-preserving surgery or by a wait-and-see policy includes the high risk of residual tumor or local tumor recurrence, which has proved to have the worst prognosis of all. As organ-preserving therapy was recommended as the method of choice, mainly to avoid anterior pelvic exenteration and urinary diversion, the question of advantages in comparison to the risk of tumor recurrence is still open. The routine use of chemoradiotherapy and increasing follow-up has called attention to a variety of chronic sequelae, such as contracted bladders with reduced capacity and upper urinary tract deterioration, which subsequently require secondary urinary diversion. Progress in continent urinary diversion may be an alternative procedure for better life quality.(ABSTRACT TRUNCATED AT 250 WORDS)