Urogenital rhabdomyosarcomas in childhood:: An interdisciplinary approach

Introduction: The survival rate of urogenital rhabdomyosarcoma (RMS) in children has dramatically improved as a result of new therapeutic strategies over the last 30 years. We present our experience, documenting different aspects of RMS therapy. Methods: From January 1980 to December 2000, 13 patients with urogenital RMS were treated in our department. Different diagnostic and therapeutic strategies in three cases are presented and discussed. Results: A metastatic alveolar RMS stage IV (IRS) was diagnosed in a 16-year-old boy. Despite different chemotherapy cycles with high dose therapy and with contemporary complete remission, the boy died 2 years after primary diagnosis of the tumor. A 15-year-old patient with a localized alveolar RMS of the small pelvis received primary neoadjuvant chemotherapy. Because of residual tumor masses, radical surgery including a colostomy was performed. This patient has been free of progression for over 7 years. In the third patient, a 3-year-old girl, paravesical embryonic RMS with involvement of the bladder was diagnosed incidentally. Despite several bladder-sparing surgical interventions, chemotherapy and radiation therapy, several local recurrences occurred. Even a salvage exenteration did not stop the local tumor progression. The female patient died 7 years after her primary diagnosis. Conclusion: In case of primary inoperability (RO resection without organ salvage is not possible) of histologically diagnosed RMS, neoadjuvant chemotherapy and/or radiation therapy is indicated. Residual tumor masses should be resected even in case of organ salvage with possible loss of life quality. Cystectomy, especially with continent diversion, is a well-established procedure with tolerable loss of quality of life and should no longer be described as mutilation. Only by combining radical surgery with effective chemotherapy is it possible to achieve long-term remission. Delayed radical surgical intervention can lead to uncontrollable tumor progression with lethal sequelae. In order to optimize therapy and because of the small number of cases, central documentation with a uniform treatment regimen for all patients should be utilized, analogous to the Cooperative Soft-Tissue-Sarcoma Study (CWS-96).