Conservative treatment for girls with nonmetastatic rhabdomyosarcoma of the genital tract: A report from the study committee of the International Society of Pediatric Oncology

Purpose: To report the results of a conservative multimodal approach in gir ls 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 t ract (vulva, vagina, uterus) were treated in SIOP protocols. With the excep tion of patients with rare small tumors, which were resected at the start o f the studies, all patients received initial chemotherapy (CHT) (ifosfamide , vincristine, and actinomycin D), Local treatment including surgery, brach ytherapy (BT), and external-beam radiotherapy (ERT) war given only to girls who did not achieve complete remission (CR) with CHT or who subsequently r elapsed. Results: The primary tumor originated in the vulva or vagina in 27 girls an d 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 foll ow-up of 5 years, 30 girls were alive and in first CR and five were alive a nd in second CR. Four patients treated with complete resection of the tumor at diagnosis received less CHT, Thirteen patients were treated with CHT al one. In 17 patients, local treatment was necessary to achieve complete loca l 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 wets radiotherapy (RT) (ERT in three patients, BT in seven), radical surgery with uterine ablation (three patients), RT and radi cal surgery (three patients), and conservative surgery with RT (one patient ). Conclusion: Girls with nonmetastatic RMS of the genital tract have an excel lent prognosis. We found no difference in outcome between uterine and vulvo vaginal 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 a lternative to radical surgery or ERT. (C) 1999 by American Society of Clini cal Oncology.