title: Clinical staging and treatment results in rhabdomyosarcoma of the female genital tract among children and adolescents.
abstract: From 1972 to 1984, 47 children and adolescents with primary tumors of the female genital tract were treated with eight different Intergroup Rhabdomyosarcoma Study (IRS I-II) protocols. These included patients with vaginal (28), uterine (ten), or vulval (nine) rhabdomyosarcoma or undifferentiated sarcomas. The mean age of patients with primary vaginal tumors was younger than 2 years. All were of the embryonal histologic subtype. The majority of these patients were treated with initial chemotherapy (vincristine, dactinomycin, +/- cyclophosphamide, +/- Adriamycin [(ADR) doxorubicin]) followed by delayed hysterectomy and/or partial vaginectomy. Among 26 patients with localized vaginal tumors, there have been six relapses resulting in one tumor-related death and one therapy-related death. The five patients with nonfatal relapse have been disease-free for from 2.5 to 6.5 years (mean, 4.4 years) since salvage therapy was commenced. The 19 patients without relapse have been continuously disease-free for 1.5 to 12 years (mean, 5.34 years: median, 6 years). No patient that received ADR initially relapsed. Ten patients, with a mean age greater than 13 years, had primary uterine sarcomas and were treated by the same chemotherapy regimens. Six with polypoid localized lesions, removed before chemotherapy, have remained disease-free for 2.5 to 6.5 years. Four, with more extensive local lesions or disseminated disease, treated with chemotherapy only, died 2 to 11 months from diagnosis. Nine patients with rhabdomyosarcomas of the vulva (age, 1-19 years) were managed by the same chemotherapy regimens +/- radiotherapy. Resection was carried out initially or after chemotherapy. Eight of these have been disease-free from 4 to 10 years (mean, 6.4 years); and one is alive with probable disease at 2.5 years.
authors.affiliation: Childrens Hospital of Los Angeles, CA 90054-0700.
authors.forename: D M
authors.initials: DM
authors.lastname: Hays
authors.forename: H
authors.initials: H
authors.lastname: Shimada
authors.forename: R B
authors.initials: RB
authors.lastname: Raney
authors.forename: M
authors.initials: M
authors.lastname: Tefft
authors.forename: W
authors.initials: W
authors.lastname: Newton
authors.forename: W M
authors.initials: WM
authors.lastname: Crist
authors.forename: W
authors.initials: W
authors.lastname: Lawrence
authors.forename: A
authors.initials: A
authors.lastname: Ragab
authors.forename: M
authors.initials: M
authors.lastname: Beltangady
authors.forename: H M
authors.initials: HM
authors.lastname: Maurer
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005069:Evaluation Studies as Topic; D005260:Female; D005833:Genital Neoplasms, Female / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery; D006801:Humans; D007044:Hysterectomy; D007223:Infant; D007231:Infant, Newborn; D009364:Neoplasm Recurrence, Local; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery; D012509:Sarcoma / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D014621:Vagina / Q000601:surgery