Abstract

Abstract Background Ewing’s sarcoma is the second most common primary malignant bone cancer in children and adolescents. Outcome of the children with Ewing sarcoma (ES) is worse in Low and Middle Income Countries (LMIC) compared to High Income Countries (HIC). In HIC only factor that had a significant impact on outcome was the presence of metastasis at presentation whereas treatment delay is a recognized factor contributing to poorer outcome evident by the studies especially done in LMIC. The situation in Sri Lanka has not been described before. We analyzed the clinical features and the impact of treatment delays on 5 year Event Free Survival (EFS) in Sri Lanka. Method All children with histologically proven Ewing sarcoma registered at the paediatric department from 01.01.2006 to 31.12.2011 were included. Demographic data, dates of first registration,chemotherapy, surgery, last contact and date of an event (Death, recurrence, progressive disease)were extracted from clinic records. EFS was calculated with Kaplan Meir curves and log rank test were used to calculate the level of significance. Results Out of 49 patients commonest site of presentation was leg (n = 19) followed by chest wall (n = 16). 10% had metastasis (n = 5) at presentation. 65% (n = 32) had surgery as local treatment (LT) and rest had radiotherapy while 4 defaulted treatment before LT. Out of extremity tumours only 21% (5/23) had limb preservation surgery. 5 year EFS in the entire cohort were 23.8%. Mean delay in neoadjuvant chemotherapy (NAC), LT and adjuvant chemotherapy (AC) were 6, 5.59 and 9.69 weeks respectively. NAC (p = 0.035) and LT(p = 0.004) delays significantly impacted EFS. Conclusions In spite of demographic and pathological data are similar to published data, 5 year EFS was is inferior to the accepted international standards. Delays in NAC and LT is the likely reason for inferior outcome.

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