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

Author:

Pinto Alfredo1,Dickman Paul23,Parham David4

Affiliation:

1. Calgary Laboratory Services, University of Calgary, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, Canada T3B 6A8

2. Department of Pathology, Phoenix Children's Hospital, 1919 E. Thomas Road, Phoenix, AZ 85016, USA

3. Departments of Pathology and Pediatrics, University of Arizona, College of Medicine, Phoenix, AZ 85016, USA

4. Health Sciences Center, University of Oklahoma, Oklahoma City, OK 73104, USA

Abstract

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.

Publisher

Hindawi Limited

Subject

Radiology, Nuclear Medicine and imaging,Oncology

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