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Pelvic Rhabdomyosarcomas in Childhood

The diagnosis of pelvic rhabdomyosarcoma in childhood may ordinarily be established without laparotomy. A search for dissemination is rarely positive at the time of discovery of the tumor. Regimens of combined therapy including 1) radical surgical excision, 2) pelvic irradiation, and 3) multiple‐agent long‐range chemotherapy have resulted in survival rates of 60–90% in children with apparently localized disease. Satisfaction with the results of such regimens, relative to survival, is offset by the adverse effects of the exenteration procedures required and to a lesser extent by the morbidity associated with pelvic irradiation. New approaches have been developed in an attempt to avoid exenteration and radiotherapy. These include limited local surgery, i.e., partial cystectomy or tumorectomy, in rhabdomyosarcomas of the bladder, for example. The results of these procedures have been markedly better than those that followed the use of similar, limited operations before the use of effective chemotherapy. More radical departures from accepted tumor management, i.e., primary chemotherapy regimens which do not include initial surgery (except biopsy) and which may or may not include radiotherapy, have also been employed. In approximately 70% of infants and small children with pelvic rhabdomyosarcoma, tumor dissemination can be controlled by an approach that first uses only chemotherapy. On this regimen, a majority of these neoplasms will be reduced in size to the extent that a limited surgical excision is possible, or they may be treated without excisional surgery.