MR imaging features of diffuse intrinsic pontine glioma and relationship to overall survival: Report from the International DIPG Registry

James L. Leach*, James Roebker, Austin Schafer, Joshua Baugh, Brooklyn Chaney, Christine Fuller, Maryam Fouladi, Adam Lane, Renee Doughman, Rachid Drissi, Mariko DeWire-Schottmiller, David S. Ziegler, Jane E. Minturn, Jordan R. Hansford, Stacie S. Wang, Michelle Monje-Deisseroth, Paul G. Fisher, Nicholas G. Gottardo, Hetal Dholaria, Roger PackerKatherine Warren, Sarah E.S. Leary, Stewart Goldman, Ute Bartels, Cynthia Hawkins, Blaise V. Jones

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

Background. This study describes imaging features of diffuse intrinsic pontine glioma (DIPG) and correlates with overall survival (OS) and histone mutation status in the International DIPG Registry (IDIPGR). Methods. Four hundred cases submitted to the IDIPGR with a local diagnosis of DIPG and baseline MRI were evaluated by consensus review of 2 neuroradiologists; 43 cases were excluded (inadequate imaging or alternative diagnoses). Agreement between reviewers, association with histone status, and univariable and multivariable analyses relative to OS were assessed. Results. On univariable analysis imaging features significantly associated with worse OS included: extrapontine extension, larger size, enhancement, necrosis, diffusion restriction, and distant disease. On central review, 9.5% of patients were considered not to have DIPG. There was moderate mean agreement of MRI features between reviewers. On multivariable analysis, chemotherapy, age, and distant disease were predictors of OS. There was no difference in OS between wild-type and H3 mutated cases. The only imaging feature associated with histone status was the presence of ill-defined signal infiltrating pontine fibers. Conclusions. Baseline imaging features are assessed in the IDIPGR. There was a 9.5% discordance in DIPG diagnosis between local and central review, demonstrating need for central imaging confirmation for prospective trials. Although several imaging features were significantly associated with OS (univariable), only age and distant disease were significant on multivariable analyses. There was limited association of imaging features with histone mutation status, although numbers are small and evaluation exploratory.

Original languageEnglish (US)
Pages (from-to)1647-1657
Number of pages11
JournalNeuro-oncology
Volume22
Issue number11
DOIs
StatePublished - Nov 1 2020

Keywords

  • DIPG
  • Diffuse midline glioma
  • Histone mutation
  • International DIPG registry
  • MRI

ASJC Scopus subject areas

  • Oncology
  • Clinical Neurology
  • Cancer Research

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