Evaluation of outcome and prognostic factors in paediatric extraskeletal ewings sarcoma: A single institution experience.

Abstract

e21507 Background: Ewing's sarcoma (ES) represent the most frequent small and round cell bone tumors of childhood and adolescence. Data on extraskeletal ewings sarcoma (EES) with uniform chemotherapy protocol are very minimal. We aimed to assess this aspect in our patients and identify prognostic factors in patients with extraskeletal ewings sarcoma. Methods: A retrospective analysis of data was done between January 2005 and January 2016. These paediatric EES patients who received chemotherapy with VAC-IE regimen were evaluated for their response, overall survival (OS) and Event free survival (EFS). The prognostic factors which influenced their overall survival were also analysed. Results: Fourty one paediatric EES patients were diagnosed at our institute out of which 31 patients received treatment at our hospital. 25.8% (8) were metastatic at presentation. The mean age was 12.37 years (1-18 years) with a predominant female preponderance 54.8%. The commonest site at presentation was presacral mass (7) and mass in the brain parenchyma (8). Rare sites of presentation as a nasal mass and parotid mass was seen in one patients each. 78% received chemotherapy with VAC-IE regimen. The ORR was 71% (8 CR + 9 PR). Toxicity was grade III neutropenia in 5 (20.8%). 8 patients received local RT. The 3 year OS and EFS in the localized EES were 55% and 39% and in metastatic EES were 35% and 24% respectively. Low hemoglobin (P = 0.05) and high LDH (P = 0.01) predicted inferior OS for the entire EES cohort on multivariate analysis. Conclusions: High LDH and low hemoglobin is associated with poor OS for patients with EES ewings sarcoma. VAC-IE is a very effective and a safe chemotherapy regimen and should be considered in patients with extraskeletal ewings sarcoma.