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Volume 2011, Article ID 856190, 15 pages
Review Article

Pathobiologic Markers of the Ewing Sarcoma Family of Tumors: State of the Art and Prediction of Behaviour

1Calgary Laboratory Services, University of Calgary, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, Canada T3B 6A8
2Department of Pathology, Phoenix Children's Hospital, 1919 E. Thomas Road, Phoenix, AZ 85016, USA
3Departments of Pathology and Pediatrics, University of Arizona, College of Medicine, Phoenix, AZ 85016, USA
4Health Sciences Center, University of Oklahoma, Oklahoma City, OK 73104, USA

Received 6 July 2010; Revised 20 September 2010; Accepted 23 September 2010

Academic Editor: R. Pollock

Copyright © 2011 Alfredo Pinto 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.


Over the past three decades, the outcome of Ewing sarcoma family tumor (ESFT) patients who are nonmetastatic at presentation has improved considerably. The prognosis of patients with metastatic disease at the time of diagnosis and recurrence after therapy remains dismal. Drug-resistant disease at diagnosis or at relapse remains a major cause of mortality among patients diagnosed with ESFT. In order to improve the outcome for patients with potential relapse, there is an urgent need to find reliable markers that either predict tumor behaviour at diagnosis or identify therapeutic molecular targets at the time of recurrence. An improved understanding of the cell of origin and the molecular pathways that regulate tumorigenicity in ESFT should aid us in the search for novel therapies for ESFT. The purpose of this paper is thus to outline current concepts of sarcomagenesis in ESFT and to discuss ESFT patterns of differentiation and molecular markers that might affect prognosis or direct future therapeutic development.