title: [Targeted therapy of sarcomas].
abstract: Recent progress made in the field of sarcoma biology has shed new light on the pathophysiology of these numerous but rare diseases. Soft tissue sarcomas can be divided into 6 sub-types based on the underlying molecular biology of the disease: 1) translocation leading to fusion proteins involving transcription factors or growth factors (Ewing sarcoma, myxoid liposarcoma, dermatofibrosarcoma protuberans); 2) tyrosine kinase receptor mutations (gastrointestinal stromal tumors); 3) tumor-suppressor gene deletion (type 1 neurofibromatosis, rhabdoid tumors); 4) genetic alteration such as amplification of chromosomal regions (well differentiated/dedifferentiated liposarcoma); 5) sarcomas with more complex genetic alterations (leiomyosarcoma) and 6) abnormalities involving the cell-adhesion pathways (aggressive fibromatosis). Together with the current development of numerous targeted therapies, these recent progress are the basis of tomorrow's personalised medicine for patients with soft tissue sarcoma.
authors.affiliation: Hôpital Edouard-Herriot, Service d'oncologie médicale, Lyon, France.
authors.forename: Philippe A
authors.initials: PA
authors.lastname: Cassier
authors.forename: Armelle
authors.initials: A
authors.lastname: Dufresne
authors.forename: Hiba
authors.initials: H
authors.lastname: El Sayadi
authors.forename: Daniel
authors.initials: D
authors.lastname: Pissaloux
authors.forename: Laurent
authors.initials: L
authors.lastname: Alberti
authors.forename: Anne Valérie
authors.initials: AV
authors.lastname: Decouvelaere
authors.forename: Dominique
authors.initials: D
authors.lastname: Ranchere
authors.forename: Isabelle
authors.initials: I
authors.lastname: Ray-Coquard
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Blay
mesh_terms: D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D046152:Gastrointestinal Stromal Tumors / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D012509:Sarcoma / Q000145:classification / Q000188:drug therapy* / Q000235:genetics
chemical_list: D020533:Angiogenesis Inhibitors; D000970:Antineoplastic Agents; D047428:Protein Kinase Inhibitors