[en] Since 1975, 4 rhabdomyosarcoma (RMS) studies have been undertaken by the SIOP (RMS 75, MMT 84, MMT 89 and presently MMT 95) (FLAMANT 1985 -1997). The main goal of theses protocols was to improve the survival of children with RMS while reducing late effects from therapy by restricting the indications for local treatment (surgery and/or radiotherapy) after good response to initial chemotherapy (CT). All theses studies are based on the proven efficacy of initial CT (Vincristine, Dactinomycin, Cyclophosphamide replaced by Ifosfamide) followed by local treatment of residual disease. In case of complete clinical and radiological remission after initial CT, a Mm study (Godzinski 1994) concluded that positive biopsies were rare (5%) and that the local relapse rate remained high even when biopsies apparently confirmed complete remission. subsequently, MMT studies have not placed such emphasis on the value of second look surgery to confirm clinical and radiological evidence of remission. surgery is therefore indicated in case of residual mass after initial CT and should, as a rule, be conservative, anticipating local radiotherapy for residual disease. Genito-urinary RMS account for 24% of all RMS in MMT 84 study and 28% in MMT 89 study. They are classified into two groups, according to slop workshop (RODARY 1989): genito-urinary bladder prostate (GUBP) and genito-urinary non-bladder prostate (GU non BP) including paratesticular RMS in boys and uterine and/or vulvo-vaginal RMS in girls. According to MMT 84 study, tumor site is an important prognostic factor in multivariate analysis as well as T status (according to SIOP UICC- TNM classification) and histology (alveolar versus non alveolar). GU non-BP represents the most favourable site with orbit followed in order by GUBP, head and neck, limbs and other sites

[en] Purpose: To report the Institut Gustave Roussy brachytherapy (BT) experience in the management of vulval and vaginal rhabdomyosarcoma with special emphasis on long-term outcome. Patients and Methods: Between 1971 and 2005, the data concerning 39 girls who had undergone BT as a part of their treatment were retrospectively analyzed. Of the 39 girls, 20 had been treated before 1990, when the BT volume encompassed the initial tumor extension. After 1990, only residual disease was included in the BT volume. Side effects were classified using the Common Terminology Criteria for Adverse Events, version 3.0. Results: The median age was 16.3 months at diagnosis. Vaginal or vulvar rhabdomyosarcoma was diagnosed in 26 and 6 patients, respectively. The median follow-up was 8.4 years. The 5-year overall survival rate was 91%. Of the 39 patients, 6 developed a relapse. Of the 20 patients treated before 1990, 6 experienced Grade 1-2 renal/genitourinary function symptoms and 75% developed sequelae, in the form of vaginal or urethral sclerosis or stenosis. Four patients received follow-up treatment for psychological disorders. Of the 19 patients treated after 1990, 2 developed acute side effects, with maximal Grade 1-2 renal/genitourinary function symptoms, and 20% developed vaginal or urethral sclerosis or stenosis. Two cases of psychological disturbances were also documented. Conclusion: Reducing the BT volume coverage, better indications for surgery, and more efficient chemotherapy, all combined within a multidisciplinary approach, tended to improve results in terms of both survival and long-term sequelae