title: Novel therapeutic approaches in chondrosarcoma.
abstract: Chondrosarcoma is a malignant tumor of bones, characterized by the production of cartilage matrix. Due to lack of effective treatment for advanced disease, the clinical management of chondrosarcomas is exceptionally challenging. Current research focuses on elucidating the molecular events underlying the pathogenesis of this rare bone malignancy, with the goal of developing new molecularly targeted therapies. Signaling pathways suggested to have a role in chondrosarcoma include Hedgehog, Src, PI3k-Akt-mTOR and angiogenesis. Mutations in IDH1/2, present in more than 50% of primary conventional chondrosarcomas, make the development of IDH inhibitors a promising treatment option. The present review discusses the preclinical and early clinical data on novel targeted therapeutic approaches in chondrosarcoma.
authors.affiliation: London Sarcoma Service, University College London Hospital, London, UK.
authors.forename: Genovefa
authors.initials: G
authors.lastname: Polychronidou
authors.affiliation: London Sarcoma Service, University College London Hospital, London, UK.
authors.forename: Vasilios
authors.initials: V
authors.lastname: Karavasilis
authors.affiliation: Fred Hutchinson Cancer Research Center, Seattle, WA, USA.
authors.forename: Seth M
authors.initials: SM
authors.lastname: Pollack
authors.affiliation: Division of Cancer Biology, The Institute of Cancer Research, London, UK.
authors.forename: Paul H
authors.initials: PH
authors.lastname: Huang
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital, London, UK.
authors.forename: Alex
authors.initials: A
authors.lastname: Lee
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital, London, UK.|Division of Clinical Studies, The Institute of Cancer Research, London, UK.
authors.forename: Robin L
authors.initials: RL
authors.lastname: Jones
mesh_terms: D020533:Angiogenesis Inhibitors / Q000494:pharmacology / Q000627:therapeutic use; D000818:Animals; D000970:Antineoplastic Agents / Q000494:pharmacology / Q000627:therapeutic use*; D002813:Chondrosarcoma / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism; D019468:Disease Management; D053823:Hedgehog Proteins / Q000378:metabolism; D056572:Histone Deacetylase Inhibitors / Q000494:pharmacology / Q000627:therapeutic use; D006801:Humans; D007521:Isocitrate Dehydrogenase / Q000235:genetics / Q000378:metabolism; D058990:Molecular Targeted Therapy; D009154:Mutation; D019869:Phosphatidylinositol 3-Kinases / Q000378:metabolism; D047428:Protein Kinase Inhibitors / Q000494:pharmacology / Q000627:therapeutic use; D051057:Proto-Oncogene Proteins c-akt / Q000378:metabolism; D015398:Signal Transduction / Q000187:drug effects; D058570:TOR Serine-Threonine Kinases / Q000378:metabolism; D019061:src-Family Kinases / Q000378:metabolism
chemical_list: D020533:Angiogenesis Inhibitors; D000970:Antineoplastic Agents; D053823:Hedgehog Proteins; D056572:Histone Deacetylase Inhibitors; D007521:Isocitrate Dehydrogenase; D019869:Phosphatidylinositol 3-Kinases; D047428:Protein Kinase Inhibitors; D051057:Proto-Oncogene Proteins c-akt; D058570:TOR Serine-Threonine Kinases; D019061:src-Family Kinases
keywords: HDACIs; Hedgehog pathway; IDH1/2; Src pathway; chondrosarcoma; novel agents; treatment