Table of Contents
ISRN Oncology
Volume 2012, Article ID 609439, 9 pages
http://dx.doi.org/10.5402/2012/609439
Review Article

Targeted Therapy in Ewing Sarcoma

1Division of Pediatric Hematology and Oncology, Department of Pediatrics, University Medical Center Freiburg, 79106 Freiburg, Germany
2Division of Pediatric Hematology and Oncology, Children's National Medical Center, Washington, DC 20010, USA

Received 29 December 2011; Accepted 18 January 2012

Academic Editors: I. Faraoni, R. Nahta, and K. Sonoda

Copyright © 2012 A. Lissat et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Despite marked improvement in the prognosis of patients with nonmetastatic Ewing sarcoma (ES), the outcome for patients with recurrent or metastatic disease remains poor. Insight into key biologic processes in ES could provide new therapeutic targets. The particular biologic feature of ES, the fusion of the EWS gene with a member of the ETS family of genes, is present in >95% of cases. The EWS-ETS chimeric protein leads to aberrant transcription that promotes tumor initiation and propagation via prosurvival and antiapoptotic pathways. Recent research has identified cooperating mutations important for ES tumorigenesis. This paper provides a summary of the latest research in ES and discusses potential novel targets for therapy.