Diagnostic implications of TERT promoter mutation status in diffuse gliomas in a routine clinical setting.

IDH (isocitrate dehydrogenase) gene mutations are present in most diffuse low-grade gliomas and define the clinico-pathological core of the respective morphologically defined entities. Conversely, according to the 2016 WHO classification, the majority of glioblastomas belong to the IDH-wildtype cate...

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Publicado en:Virchows Archiv: European Journal of Pathology Vol. 471; no. 5; pp. 641 - 650
Autores principales: Hewer, Ekkehard, Prebil, Nadine, Berezowska, Sabina, Gutt-Will, Marielena, Schucht, Philippe, Dettmer, Matthias, Vassella, Erik, Dettmer, Matthias S
Formato: Journal Article
Publicado: Springer Nature Nov2017
Acceso en línea:Ver este registro en EBSCOhost
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        atl: Diagnostic implications of TERT promoter mutation status in diffuse gliomas in a routine clinical setting.
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          Hewer, Ekkehard
          Prebil, Nadine
          Berezowska, Sabina
          Gutt-Will, Marielena
          Schucht, Philippe
          Dettmer, Matthias
          Vassella, Erik
          Dettmer, Matthias S
        affil: Institute of Pathology , University of Bern , Murtenstrasse 31 3010 Bern Switzerland
      sug:
        subj:
          Glioma
          Transferases
          Brain Neoplasms
          Sequence Analysis
          Male
          Aged, 80 and Over
          Glioma Diagnosis
          Young Adult
          Genes
          Adolescence
          Child
          Brain Neoplasms Mortality
          Aged
          Middle Age
          Mutation
          Female
          Adult
          Infant
          Glioma Mortality
          Brain Neoplasms Diagnosis
          Kaplan-Meier Estimator
          Scales
          Aged, 80 & over
          Adolescent: 13-18 years
          Child: 6-12 years
          Aged: 65+ years
          Middle Aged: 45-64 years
          Adult: 19-44 years
          Infant: 1-23 months
          Male
          Female
      ab: IDH (isocitrate dehydrogenase) gene mutations are present in most diffuse low-grade gliomas and define the clinico-pathological core of the respective morphologically defined entities. Conversely, according to the 2016 WHO classification, the majority of glioblastomas belong to the IDH-wildtype category, which is defined by exclusion. TERT (telomerase reverse transcriptase gene) promoter mutations have been suggested as a molecular marker for primary glioblastomas. We analyzed molecular, histopathological, and clinical profiles of a series of 110 consecutive diffuse gliomas (WHO grades II-IV) diagnosed at our institution, in which TERT promoter mutation analysis had been performed as part of diagnostic work-up. A diagnostic algorithm based on IDH, TERT, ATRX, H3F3A, and 1p19q co-deletion status resulted in a consistent molecular classification with only 14 (13%) marker-negative tumors. TERT promoter mutations were present in 77% of IDH-wildtype tumors. The TERT/IDH-wildtype category was highly enriched for tumors with unconventional clinical or histological features. Molecular classes were associated with distinct rates of MGMT promoter methylation. We conclude that, in a routine diagnostic setting, TERT promoter mutations define a relatively homogeneous core group among IDH-wildtype diffuse gliomas that includes the majority of primary glioblastomas as well as their putative precursor lesions.
      pubtype: Academic Journal
      doctype: Journal Article
      ougenre: Article
    language: English
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