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Volume 2012, Article ID 301975, 18 pages
Review Article

Bone Sarcomas: From Biology to Targeted Therapies

1Department of Oncology for Children and Adolescents, Institut Gustave Roussy, 114 rue Edouard Vaillant, 94805 Villejuif Cedex, France
2Inserm U957-EA 3822, Faculté de Médecine, 1 rue Gaston Veil, 44035 Nantes Cedex 1, France
3Oncopediatric Departement, CHU de Nantes, Boulevard Jacques Monod, 44093 Nantes, France
4Pediatric Onco-Hematology Department, EA 4438UdS, CHRU Strasbourg, Avenue Molière, 67000 Strasbourg, France
5Unité 830 INSERM, Institut Curie, Centre de Recherche, 26 rue d’Ulm, 75248 Paris Cedex 05, France
6Oncopediatric Departement, Centre Léon Bérard, 28 rue Laennec, 69008 Lyon, France
7Oncopediatric Departement, Hôpital La Timone, 264 rue Saint Pierre, 13385 Marseille Cedex 5, France
8Department of Medical Oncology, Institut Curie, 26 rue d’Ulm, 75248 Paris Cedex 05, France

Received 1 July 2012; Accepted 10 October 2012

Academic Editor: R. Pollock

Copyright © 2012 Nathalie Gaspar 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.


Primary malignant bone tumours, osteosarcomas, and Ewing sarcomas are rare diseases which occur mainly in adolescents and young adults. With the current therapies, some patients remain very difficult to treat, such as tumour with poor histological response to preoperative CT (or large initial tumour volume for Ewing sarcomas not operated), patients with multiple metastases at or those who relapsed. In order to develop new therapies against these rare tumours, we need to unveil the key driving factors and molecular abnormalities behind the malignant characteristics and to broaden our understanding of the phenomena sustaining the metastatic phenotype and treatment resistance in these tumours. In this paper, starting with the biology of these tumours, we will discuss potential therapeutic targets aimed at increasing local tumour control, limiting metastatic spread, and finally improving patient survival.