TERT promoter mutation confers favorable prognosis regardless of 1p/19q status in adult diffuse gliomas with IDH1/2 mutations

Acta Neuropathol Commun. 2020 Nov 23;8(1):201. doi: 10.1186/s40478-020-01078-2.

Abstract

TERT promoter mutations are commonly associated with 1p/19q codeletion in IDH-mutated gliomas. However, whether these mutations have an impact on patient survival independent of 1p/19q codeletion is unknown. In this study, we investigated the impact of TERT promoter mutations on survival in IDH-mutated glioma cases. Detailed clinical information and molecular status data were collected for a cohort of 560 adult patients with IDH-mutated gliomas. Among these patients, 279 had both TERT promoter mutation and 1p/19q codeletion, while 30 had either TERT promoter mutation (n = 24) or 1p/19q codeletion (n = 6) alone. A univariable Cox proportional hazard analysis for survival using clinical and genetic factors indicated that a Karnofsky performance status score (KPS) of 90 or 100, WHO grade II or III, TERT promoter mutation, 1p/19q codeletion, radiation therapy, and extent of resection (90-100%) were associated with favorable prognosis (p < 0.05). A multivariable Cox regression model revealed that TERT promoter mutation had a significantly favorable prognostic impact (hazard ratio = 0.421, p = 0.049), while 1p/19q codeletion did not have a significant impact (hazard ratio = 0.648, p = 0.349). Analyses incorporating patient clinical and genetic information were further conducted to identify subgroups showing the favorable prognostic impact of TERT promoter mutation. Among the grade II-III glioma patients with a KPS score of 90 or 100, those with IDH-TERT co-mutation and intact 1p/19q (n = 17) showed significantly longer survival than those with IDH mutation, wild-type TERT, and intact 1p/19q (n = 185) (5-year overall survival, 94% and 77%, respectively; p = 0.032). Our results demonstrate that TERT promoter mutation predicts favorable prognosis independent of 1p/19q codeletion in IDH-mutated gliomas. Combined with its adverse effect on survival among IDH-wild glioma cases, the bivalent prognostic impact of TERT promoter mutation may help further refine the molecular diagnosis and prognostication of diffuse gliomas.

Keywords: 1p/19q codeletion; CDKN2A; Glioma; IDH1/2; TERT.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Astrocytoma / genetics
  • Astrocytoma / pathology
  • Astrocytoma / therapy
  • Brain Neoplasms / genetics*
  • Brain Neoplasms / pathology
  • Brain Neoplasms / therapy
  • Chromosome Deletion*
  • Chromosomes, Human, Pair 1*
  • Chromosomes, Human, Pair 19*
  • Female
  • Glioblastoma / genetics
  • Glioblastoma / pathology
  • Glioblastoma / therapy
  • Glioma / genetics*
  • Glioma / pathology
  • Glioma / therapy
  • Humans
  • Isocitrate Dehydrogenase / genetics
  • Karnofsky Performance Status
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Mutation
  • Neoplasm Grading
  • Neurosurgical Procedures
  • Oligodendroglioma / genetics
  • Oligodendroglioma / pathology
  • Oligodendroglioma / therapy
  • Prognosis
  • Promoter Regions, Genetic / genetics*
  • Proportional Hazards Models
  • Radiotherapy, Adjuvant
  • Retrospective Studies
  • Survival Rate
  • Telomerase / genetics*
  • Young Adult

Substances

  • IDH2 protein, human
  • Isocitrate Dehydrogenase
  • IDH1 protein, human
  • TERT protein, human
  • Telomerase