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Volume 2011, Article ID 402508, 13 pages
Review Article

Targeting the Insulin-Like Growth Factor 1 Receptor in Ewing's Sarcoma: Reality and Expectations

1Sarcoma Unit, The Royal Marsden NHS Foundation Trust, London SW3 6JJ, UK
2Sarcoma Molecular Pathology Team, The Institute of Cancer Research, Sutton SM2 5N6, UK
3Drug Development Unit, The Royal Marsden NHS Foundation Trust, Downs Road, Sutton SM2 5PT, UK
4Fred Hutchinson Cancer Research Center, Seattle, WA 95109-4433, USA

Received 1 July 2010; Revised 19 January 2011; Accepted 8 February 2011

Academic Editor: Alberto Pappo

Copyright © 2011 David Olmos 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.


Ewing's sarcoma family of tumours comprises a group of very aggressive diseases that are potentially curable with multimodality treatment. Despite the undoubted success of current treatment, approximately 30% of patients will relapse and ultimately die of disease. The insulin-like growth factor 1 receptor (IGF-1R) has been implicated in the genesis, growth, proliferation, and the development of metastatic disease in Ewing's sarcoma. In addition, IGF1-R has been validated, both in vitro and in vivo, as a potential therapeutic target in Ewing's sarcoma. Phase I studies of IGF-1R monoclonal antibodies reported several radiological and clinical responses in Ewing's sarcoma patients, and initial reports of several Phase II studies suggest that about a fourth of the patients would benefit from IGF-1R monoclonal antibodies as single therapy, with approximately 10% of patients achieving objective responses. Furthermore, these therapies are well tolerated, and thus far severe toxicity has been rare. Other studies assessing IGF-1R monoclonal antibodies in combination with traditional cytotoxics or other targeted therapies are expected. Despite, the initial promising results, not all patients benefit from IGF-1R inhibition, and consequently, there is an urgent need for the identification of predictive markers of response.