Clinical staging and treatment results in rhabdomyosarcoma of the female genital tract among children and adolescents

Cancer. 1988 May 1;61(9):1893-903. doi: 10.1002/1097-0142(19880501)61:9<1893::aid-cncr2820610929>3.0.co;2-9.

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.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Evaluation Studies as Topic
  • Female
  • Genital Neoplasms, Female / drug therapy
  • Genital Neoplasms, Female / pathology*
  • Genital Neoplasms, Female / radiotherapy
  • Genital Neoplasms, Female / surgery
  • Humans
  • Hysterectomy
  • Infant
  • Infant, Newborn
  • Neoplasm Recurrence, Local
  • Rhabdomyosarcoma / drug therapy
  • Rhabdomyosarcoma / pathology*
  • Rhabdomyosarcoma / radiotherapy
  • Rhabdomyosarcoma / surgery
  • Sarcoma / drug therapy
  • Sarcoma / pathology
  • Sarcoma / radiotherapy
  • Sarcoma / surgery
  • Vagina / surgery