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Innovative Therapies in Ewing Sarcoma

Amaral, Ana Teresa BSc*; Ordóñez, José Luis PhD*; Otero-Motta, Ana Pastora PhD*; García-Domínguez, Daniel J. PhD*; Sevillano, María Victoria MLT*; de Álava, Enrique MD, PhD*,†

Advances in Anatomic Pathology: January 2014 - Volume 21 - Issue 1 - p 44–62
doi: 10.1097/PAP.0000000000000003
Review Articles

Ewing Sarcoma is a developmental tumor characterized by balanced chromosomal translocations and formation of new fusion genes, which are the main hallmark of this rare entity. Despite the vast knowledge regarding the molecular aspects of this rare malignancy obtained in the last few years, including the discovery of new therapeutic targets, many questions still remain open. In this review we focus on the research on targeted therapies in this malignancy, and discussed some bottlenecks related to this such as the possible role of pathologists, the availability of samples, the lack of appropriate animal models, and the resources needed to carry out preclinical and clinical research.

*Molecular Pathology Program, Institute of Biomedical Research of Salamanca-Centro de Investigación del Cáncer, Centro de Investigación del Cancér (IBSAL-CIC), Campus Miguel de Unamuno S/N, Salamanca

Pathology Department, Hospital Universitario Virgen del Rocio-IBiS, Sevilla, Spain

All figures can be viewed online in color at

A.T.A. and J.L.O. contributed equally.

The Centro de Investigación del Cáncer participates in European Clinical trials in Rare Sarcomas within an integrated translational trial network (FP7-HEALTH-2011-2-stage, Project ID 278742 EUROSARC) European Commission and PROspective VAlidation of Biomarkers in Ewing Sarcoma for personalized translational medicine (PROVABES). Work in the Molecular Pathology laboratory is supported by the ISCIII (FIS-FEDER) (PI11/00018) and PROVABES (PI12/03102), CSIC (Contratos JAE-DOC to JLOG), the Fundação para a Ciência e Tecnologia, Ministério para a Investigaçãoe Tecnologia, Portugal (ATA fellowship SFRH/BD/69318/2010), Fundación Memoria de D. Manuel Solorzano Barruso, Fundación Cris contra el cancer, and Fundación María García Estrada.

The authors have no conflicts of interest to disclose.

Reprints: Enrique de Álava, MD, PhD, Molecular Pathology Program, Institute of Biomedical Research of Salamanca-Centro de Investigación del Cáncer (IBSAL-CIC), Centro de Investigación del Cáncer- Instituto de Biología Molecular y Celular del Cáncer (IBMCC) (IBSAL-CIC), Campus Miguel de Unamuno S/N, 37007 Salamanca, Spain (e-mail:

© 2014 by Lippincott Williams & Wilkins.