Conservative Treatment for Girls With Nonmetastatic Rhabdomyosarcoma of the Genital Tract: A Report From the Study Committee of the International Society of Pediatric Oncology
- 1 July 1999
- journal article
- review article
- Published by American Society of Clinical Oncology (ASCO) in Journal of Clinical Oncology
- Vol. 17 (7), 2117
- https://doi.org/10.1200/jco.1999.17.7.2117
Abstract
PURPOSE: To report the results of a conservative multimodal approach in girls with nonmetastatic rhabdomyosarcoma (RMS) of the genital tract, treated in International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumors 84 and 89 protocols. PATIENTS AND METHODS: From 1984 to 1994, 38 girls with RMS of the genital tract (vulva, vagina, uterus) were treated in SIOP protocols. With the exception of patients with rare small tumors, which were resected at the start of the studies, all patients received initial chemotherapy (CHT) (ifosfamide, vincristine, and actinomycin D). Local treatment including surgery, brachytherapy (BT), and external-beam radiotherapy (ERT) was given only to girls who did not achieve complete remission (CR) with CHT or who subsequently relapsed. RESULTS: The primary tumor originated in the vulva or vagina in 27 girls and in the uterus in 11. The overall survival rate (± SE) was 91% ± 6% at 5 years, and the event-free survival rate was 78% ± 7%. At a median follow-up of 5 years, 30 girls were alive and in first CR and five were alive and in second CR. Four patients treated with complete resection of the tumor at diagnosis received less CHT. Thirteen patients were treated with CHT alone. In 17 patients, local treatment was necessary to achieve complete local control, for a residual mass after initial CHT (10 patients), for viable tumor on biopsy (three patients), or for local relapse (four patients). The local treatment used was radiotherapy (RT) (ERT in three patients, BT in seven), radical surgery with uterine ablation (three patients), RT and radical surgery (three patients), and conservative surgery with RT (one patient). CONCLUSION: Girls with nonmetastatic RMS of the genital tract have an excellent prognosis. We found no difference in outcome between uterine and vulvovaginal RMS. Local treatment does not seem necessary in patients who have a complete response to CHT. When a local treatment is needed, BT may be an alternative to radical surgery or ERT.This publication has 17 references indexed in Scilit:
- Treatment of non-metastatic rhabdomyosarcomas in childhood and adolescence. Results of the second study of the International Society of Paediatric Oncology: MMT84European Journal of Cancer, 1998
- Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification-an intergroup rhabdomyosarcoma studyCancer, 1995
- Conservative surgical management of vaginal and vulvar pediatric rhabdomyosarcoma: A report from the intergroup rhabdomyosarcoma study IIIJournal of Pediatric Surgery, 1995
- Conservative management of uterine pediatric rhabdomyosarcoma: A report from the intergroup rhabdomyosarcoma study III and IV pilotJournal of Pediatric Surgery, 1995
- The Third Intergroup Rhabdomyosarcoma Study.Journal of Clinical Oncology, 1995
- Value of postchemotherapy bioptical verification of complete clinical remission in previously incompletely resected (stage I and II pT3) malignant mesenchymal tumors in children: International society of pediatric oncology 1984 malignant mesenchymal tumors studyMedical and Pediatric Oncology, 1994
- Long-term sequelae of conservative treatment by surgery, brachytherapy, and chemotherapy for vulval and vaginal rhabdomyosarcoma in children.Journal of Clinical Oncology, 1990
- An attempt to use a common staging system in rhabdomyosarcoma: A report of an international workshop initiated by the international society of pediatric oncology (SIOP)Medical and Pediatric Oncology, 1989
- Prognostic factors in 281 children with nonmetastatic rhabdomyosarcoma (RMS) at diagnosisMedical and Pediatric Oncology, 1988
- Iridium afterloading curietherapy in the treatment of pediatric malignancies. The institut gustave roussy experienceCancer, 1985