title: Bone sarcomas: from biology to targeted therapies.
abstract: 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.
authors.affiliation: Department of Oncology for Children and Adolescents, Institut Gustave Roussy, 114 rue Edouard Vaillant, 94805 Villejuif Cedex, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Gaspar
authors.forename: Angela
authors.initials: A
authors.lastname: Di Giannatale
authors.forename: Birgit
authors.initials: B
authors.lastname: Geoerger
authors.forename: Françoise
authors.initials: F
authors.lastname: Redini
authors.forename: Nadège
authors.initials: N
authors.lastname: Corradini
authors.forename: Natacha
authors.initials: N
authors.lastname: Enz-Werle
authors.forename: Franck
authors.initials: F
authors.lastname: Tirode
authors.forename: Perrine
authors.initials: P
authors.lastname: Marec-Berard
authors.forename: Jean-Claude
authors.initials: JC
authors.lastname: Gentet
authors.forename: Valérie
authors.initials: V
authors.lastname: Laurence
authors.forename: Sophie
authors.initials: S
authors.lastname: Piperno-Neumann
authors.forename: Odile
authors.initials: O
authors.lastname: Oberlin
authors.forename: Laurence
authors.initials: L
authors.lastname: Brugieres