title: Molecular background of oligodendroglioma: 1p/19q, IDH, TERT, CIC and FUBP1.
abstract: Oligodendroglioma is the quintessential molecularly-defined brain tumor. The characteristic whole-arm loss of the long arm of chromosome 1 and the short arm of chromosome 19 (1p/19q-codeletion) within the genome of these tumors facilitated the reproducible molecular identification of this subcategory of gliomas. More recently, recurrent molecular genetic alterations have been identified to occur concurrently with 1p/19q-codeletion, and definitively identify these tumors, including mutations in IDH1/2, CIC, FUBP1, and the TERT promoter, as well as the absence of ATRX and TP53 alterations. These findings provide a foundation for the consistent diagnosis of this tumor type, upon which a generation of clinical investigators have assembled a strong evidence base for the effective treatment of this disease with radiation and chemotherapy.
authors.affiliation: Department of Neurosurgery & Translational Neuro-Oncology Laboratory, Harvard Medical School, Boston, MA 02115, USA.|Massachusetts General Hospital Cancer Center, Harvard Medical School, 32 Fruit Street - Yawkey 9E, Boston, MA 02114, USA.
authors.forename: Daniel P
authors.initials: DP
authors.lastname: Cahill
authors.affiliation: Massachusetts General Hospital Cancer Center, Harvard Medical School, 32 Fruit Street - Yawkey 9E, Boston, MA 02114, USA.|Department of Pathology, Harvard Medical School, Boston, MA 02115, USA.
authors.forename: David N
authors.initials: DN
authors.lastname: Louis
authors.affiliation: Southern Alberta Cancer Research Institute, University of Calgary, Calgary, AB T2N 1N4, Canada.|Hotchkiss Brain Institute, University of Calgary, Calgary, AB T2N 1N4, Canada.
authors.forename: John Gregory
authors.initials: JG
authors.lastname: Cairncross
mesh_terms: D001932:Brain Neoplasms / Q000235:genetics*; D002872:Chromosome Deletion; D002878:Chromosomes, Human, Pair 1 / Q000235:genetics; D002888:Chromosomes, Human, Pair 19 / Q000235:genetics; D004265:DNA Helicases / Q000235:genetics; D004268:DNA-Binding Proteins / Q000235:genetics; D019468:Disease Management; D057890:Epigenomics; D006801:Humans; D007521:Isocitrate Dehydrogenase / Q000235:genetics; D009154:Mutation / Q000235:genetics*; D009837:Oligodendroglioma / Q000235:genetics* / Q000628:therapy; D016601:RNA-Binding Proteins; D012097:Repressor Proteins / Q000235:genetics; D019098:Telomerase / Q000235:genetics
chemical_list: C498610:CIC protein, human; D004268:DNA-Binding Proteins; C492258:FUBP1 protein, human; D016601:RNA-Binding Proteins; D012097:Repressor Proteins; D007521:Isocitrate Dehydrogenase; C509186:TERT protein, human; D019098:Telomerase; D004265:DNA Helicases
keywords: 1p/19q loss; CIC mutation; FUBP1 mutation; IDH mutation; TERT mutation; oligodendroglioma