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.
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.
Authors: Israel Fernandez-Pineda; Sheri L Spunt; Lalit Parida; Matthew J Krasin; Andrew M Davidoff; Bhaskar N Rao Journal: J Pediatr Surg Date: 2011-11 Impact factor: 2.545
Authors: John Breneman; Jane Meza; Sarah S Donaldson; R Beverly Raney; Suzanne Wolden; Jeff Michalski; Fran Laurie; David A Rodeberg; William Meyer; David Walterhouse; Douglas S Hawkins Journal: Int J Radiat Oncol Biol Phys Date: 2011-11-19 Impact factor: 7.038
Authors: Veronique Minard-Colin; David Walterhouse; Gianni Bisogno; Helene Martelli; James Anderson; David A Rodeberg; Andrea Ferrari; Meriel Jenney; Suzanne Wolden; Gianluca De Salvo; Carola Arndt; Johannes H M Merks; Soledad Gallego; Dominique Schwob; Christine Haie-Meder; Christophe Bergeron; Michael C G Stevens; Odile Oberlin; Douglas Hawkins Journal: Pediatr Blood Cancer Date: 2018-05-21 Impact factor: 3.167
Authors: David O Walterhouse; Jane L Meza; John C Breneman; Sarah S Donaldson; Andrea Hayes-Jordan; Alberto S Pappo; Carola Arndt; R Beverly Raney; William H Meyer; Douglas S Hawkins Journal: Pediatr Blood Cancer Date: 2011-02-04 Impact factor: 3.167
Authors: Susanne E M Gruessner; Charles O A Omwandho; Thomas Dreyer; Renate Blütters-Sawatzki; Alfred Reiter; Hans R Tinneberg; Rainer M Bohle Journal: Eur J Pediatr Date: 2004-06-02 Impact factor: 3.183