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.