title: Ewing's sarcoma of the axial system in patients older than 15 years: dismal prognosis despite intensive multiagent chemotherapy and aggressive local treatment.
abstract: BACKGROUND
  Older age and axial location of Ewing's sarcoma have been reported as unfavorable prognostic factors.
  
  
  METHODS
  The records of patients older than 15 years with the Ewing's family of tumors were reviewed retrospectively. After the induction chemotherapy consisting of alternating vincristine, adriablastin, cyclophosphamide (VAC) and etoposide, ifosfamide with mesna protection (IE), a local treatment modality was chosen based on tumor and patient characteristics.
  
  
  RESULTS
  Twenty-five patients with a median age of 19 years were evaluated. Median follow-up was 26 months (range 4-58). Seventeen patients (68%) had died. In univariate analysis, factors predictive of shorter survival were the patients presenting with metastatic disease, with the primary tumor located at the pelvis, those who never achieved complete response to chemotherapy and those who had chemotherapy for <12 months. Only a negative link with pelvic location was observed in multivariate analysis [risk ratio 7.5; 95% confidence interval (CI) 1.52-37.06; P = 0.0134]. Median progression-free survival (PFS) and overall survival (OS) were 10 months (95% CI 6.2-13.8) and 14 months (95% CI 9.3-18.7), respectively. Cumulative 2-year PFS and OS were 19.0% (95% CI, SD +/-8.4) and 32.7% (95% CI, SD +/-9.8), respectively.
  
  
  CONCLUSIONS
  The prognosis of patients with axial Ewing's sarcoma is dismal despite an intensive, multimodality approach including multiagent, alternating chemotherapy, surgery and/or radiotherapy. A more aggressive approach should be considered for this group of Ewing's sarcoma patients.
authors.affiliation: Medical Oncology Department, Istanbul Medical Faculty, University of Istanbul, Istanbul, Turkey. argona@ttnet.net.tr
authors.forename: Andac
authors.initials: A
authors.lastname: Argon
authors.forename: Mert
authors.initials: M
authors.lastname: Basaran
authors.forename: Fulya
authors.initials: F
authors.lastname: Yaman
authors.forename: Yavuz
authors.initials: Y
authors.lastname: Dizdar
authors.forename: Burak
authors.initials: B
authors.lastname: Sakar
authors.forename: Hakan
authors.initials: H
authors.lastname: Camlica
authors.forename: Sevil E
authors.initials: SE
authors.lastname: Bavbek
authors.forename: Harzem
authors.initials: H
authors.lastname: Ozger
authors.forename: Emin
authors.initials: E
authors.lastname: Darendeliler
authors.forename: Haluk
authors.initials: H
authors.lastname: Onat
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000188:drug therapy* / Q000401:mortality; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D004334:Drug Administration Schedule; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D008297:Male; D015999:Multivariate Analysis; D011379:Prognosis; D012189:Retrospective Studies; D012512:Sarcoma, Ewing / Q000188:drug therapy* / Q000401:mortality; D015996:Survival Rate; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D007069:Ifosfamide