Vulval and vaginal rhabdomyosarcoma in children: update and reappraisal of Institut Gustave Roussy brachytherapy experience.

摘要

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.