title: What constitutes optimal therapy for patients with rhabdomyosarcoma of the female genital tract?
abstract: BACKGROUND
  Factors affecting outcome for rhabdomyosarcoma (RMS) of the female genital tract in patients treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols I-IV were evaluated to define optimal therapy.
  
  
  METHODS
  Records of 151 patients with tumors of the female genital tract who were treated on IRSG protocols I-IV were reviewed for details regarding chemotherapy, surgery, radiotherapy (RT), and outcome.
  
  
  RESULTS
  The overall 5-year survival was 82%, (87% for patients with locoregional tumors). Chemotherapy was primarily vincristine, actinomycin-D, and cyclophosphamide (VAC) based. Local therapy was surgery alone in 42% of patients, surgery plus RT in 19% of patients, biopsy plus RT in 12% of patients, and biopsy without RT in 21% of patients. The rate of hysterectomy decreased from 48% in IRS-I/II to 22% in IRS-III/IV with an increase in the use of RT from 23% in IRS-II to 45% in IRS-IV and continued excellent survival. Many patients with vaginal primary tumors received delayed RT or had it omitted on later studies with excellent outcome. For patients with localized embryonal/botryoid tumors, there were no significant differences in 5-year survival among patients with tumors at different sites or among patients treated on IRS-I-IV. In patients with Group I-III tumors, 43% of deaths were from toxicity. Analysis of prognostic factors, with toxic deaths censored, revealed that an age of 1-9 years at the time of diagnosis, noninvasive tumors, and the use of IRS-II or IRS-IV treatments were associated significantly with better outcome. Patients ages 1-9 years fared best (5-year survival of 98%) and patients outside of this age range especially benefited from the intensified therapy used in IRS-III or IRS-IV (5-year survival of 67% on the IRS-I/II vs. 90% in IRS-III/IV).
  
  
  CONCLUSIONS
  Localized female genital RMS usually is curable with combination chemotherapy, a conservative surgical approach, and the use of RT for selected patients.
authors: Mayo Clinic, Rochester, Minnesota, USA.
authors: C A
authors: CA
authors: Arndt
authors: S S
authors: SS
authors: Donaldson
authors: J R
authors: JR
authors: Anderson
authors: R J
authors: RJ
authors: Andrassy
authors: F
authors: F
authors: Laurie
authors: M P
authors: MP
authors: Link
authors: R B
authors: RB
authors: Raney
authors: H M
authors: HM
authors: Maurer
authors: W M
authors: WM
authors: Crist
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D005260:Female; D005833:Genital Neoplasms, Female / Q000401:mortality / Q000628:therapy*; D006801:Humans; D007223:Infant; D011379:Prognosis; D012208:Rhabdomyosarcoma / Q000401:mortality / Q000628:therapy*; D015996:Survival Rate; D016896:Treatment Outcome