title: Pediatric glioblastoma with oligodendroglioma component: aggressive clinical phenotype with distinct molecular characteristics.
abstract: The 2007 World Health Organization classification defined a new variant of glioblastoma (GBM) containing oligodendroglioma foci as GBM with an oligodendroglioma component (GBMO), which shows a favorable clinical outcome compared with "classic" GBM. However, all of the reported cases of GBMO have been adult cases, with no previous reports of pediatric cases. In this report, we demonstrated molecular characteristics of a pediatric GBMO case, showing aggressive clinical behavior with 8-month overall survival. The case showed neither isocitrate dehydrogenase 1/2 genes (IDH1/2) mutation nor 1p/19q co-deletion, a hallmark of oligodendroglioal tumors. In addition, microsatellite instability, leading to the putative mechanism of temozolomide (TMZ) resistance, was frequently detected. Molecular genetic analysis may provide critical prognostic and therapeutic insights, especially for the pediatric glioma containing oligodendroglioma components.
authors.affiliation: Department of Neurosurgery, Kyushu University, Fukuoka, Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Mizoguchi
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Hata
authors.forename: Satoshi O
authors.initials: SO
authors.lastname: Suzuki
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Fujioka
authors.forename: Hideki
authors.initials: H
authors.lastname: Murata
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Amano
authors.forename: Akira
authors.initials: A
authors.lastname: Nakamizo
authors.forename: Koji
authors.initials: K
authors.lastname: Yoshimoto
authors.forename: Toru
authors.initials: T
authors.lastname: Iwaki
authors.forename: Tomio
authors.initials: T
authors.lastname: Sasaki
mesh_terms: D001932:Brain Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D059248:Chemoradiotherapy; D002648:Child; D003606:Dacarbazine / Q000031:analogs & derivatives / Q000627:therapeutic use; D017809:Fatal Outcome; D005909:Glioblastoma / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008297:Male; D053842:Microsatellite Instability; D018193:Neoplasms, Complex and Mixed / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D019635:Neurosurgical Procedures; D009837:Oligodendroglioma / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D010641:Phenotype; D000077204:Temozolomide
chemical_list: D003606:Dacarbazine; D000077204:Temozolomide
keywords: 1p/19q co-deletion; glioblastoma; microsatellite instability; oligodendroglioma; pediatric; temozolomide