BackgroundThe prognosis in patients with relapsed Ewing sarcoma is unfavorable. Our investigation identifies factors predicting for the outcome following relapse.ProcedureWe analyzed type of relapse, time to relapse and overall survival after relapse (OSr) in 714 patients with first recurrence. All patients had been treated within the Cooperative Ewing Sarcoma Studies (CESS) 81 or 86, or the European Intergroup CESS (EICESS 92). OSr time was calculated from diagnosis of first relapse to last follow‐up or death.ResultsMedian follow‐up time from diagnosis of primary disease was 2.2 years (mean = 4.0; range: 0.2–24.9). Relapse sites were local in 15%, combined local and systemic in 12%, and systemic in 73%. Among patients with a localized primary tumor, 20% relapsed locally, while 12% showed combined and 68% systemic relapse. When the primary disease was disseminated, 82% developed systemic, 13% combined, and 5% local relapse. Five‐year OSr was 0.13 (SE = 0.01). Outcome following local relapse, with a 5‐year survival rate of 0.24 (P < 0.001), was superior to outcome after systemic or combined recurrence. Five‐year OSr was 0.07 (SE = 0.01) in patients who relapsed 0–2 years after the diagnosis of primary disease, as compared to a 5‐year OSr of 0.29 (SE = 0.03) when relapse occurred later.Conclusions5‐year OSr in Ewing sarcoma is poor (<0.2). Prognostically favorable factors are: late onset (>2 years) and strictly localized relapse. Pediatr Blood Cancer 2011; 57: 549–553. © 2011 Wiley‐Liss, Inc.

BACKGROUND:The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES) was investigated.METHODS:We analyzed 120 patients registered into the European Ewing Tumor Working Initiative of National Groups (EURO‐E.W.I.N.G. 99) trial at the trial center of Muenster from 1998 to 2006. Median age was 16.2 years. Local treatment of the primary tumor was surgery in 26 of 120 patients, surgery and radiotherapy in 21 patients, and definitive radiotherapy in 40 patients. For treatment of metastases, 6 of 120 patients received surgery; 9 patients, surgery and radiotherapy; and 33 patients, definitive radiotherapy. Forty‐seven (39%) patients had local treatment of both the primary tumor and metastases, 41 (34%) patients of either the primary tumor or metastases, and 32 (27%) received no local therapy.RESULTS:Event‐free survival (EFS) at 3 years was 0.24 (95% CI, 0.16‐0.33). Univariate analyses demonstrated the impact of local therapy given to the primary tumor: 3‐year EFS was 0.25 with surgery, 0.47 with surgery and radiotherapy, 0.23 with radiotherapy, and 0.13 when no local therapy was administered (P < .001). Three‐year EFS in PDMES was also influenced by the local treatment: surgery, 0.33; surgery and radiotherapy, 0.56; radiotherapy, 0.35; no local therapy, 0.16 (P = .003). Three‐year EFS was 0.39 in patients who received local treatment of both primary tumor and PDMES, compared with 0.17 in patients with any local treatment of either primary tumor or PDMES and 0.14 in patients with no local therapy (P < .001). Multivariate analysis showed absence of local treatment to be the major risk factor (HR = 2.21; P = .027; n = 20).CONCLUSIONS:Local therapy of involved sites is important for patients with PDMES and should complement systemic treatment whenever possible. Cancer 2010. © 2010 American Cancer Society.

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Background: Ewing’s tumors (ET) are rare in patients over the age of 40 years. Published data on presentation, treatment, and clinical outcome are limited. Patients and Methods: We present a retrospective analysis of data from 47 patients in this age group diagnosed with ET and enrolled in the 2 consecutive trials, EICESS 92 and EURO-E.W.I.N.G. 99. The median age at diagnosis was 47.7 years (range, 40–68.6 years). Results: The median follow-up was 2.23 years from diagnosis (range, 0.35–12.92 years). 72.3% of patients were found to have localized disease, and 27.7% had primary metastases. Good clinical response to induction therapy was observed in 55%, and 73% of patients showed good histological response. The event-free survival was 0.77 at 1 year and 0.50 at 3 years (n = 44). Conclusion: ET are rare in patients over the age of 40 years. With adequate multimodal therapy, the results in terms of survival are comparable to those in adolescence. Specific age-adapted treatment regimens are not established. Patients should be enrolled in international trials, and if necessary treatment should be adjusted for lower tolerance and co-morbidity.