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

• Published in: Neuro-oncology (Charlottesville, Va.) Vol. 22; no. 11; pp. 1647 - 1657
• Main Authors: Leach, James L, Roebker, James, Schafer, Austin, Baugh, Joshua, Chaney, Brooklyn, Fuller, Christine, Fouladi, Maryam, Lane, Adam, Doughman, Renee, Drissi, Rachid, DeWire-Schottmiller, Mariko, Ziegler, David S, Minturn, Jane E, Hansford, Jordan R, Wang, Stacie S, Monje-Deisseroth, Michelle, Fisher, Paul G, Gottardo, Nicholas G, Dholaria, Hetal, Packer, Roger, Warren, Katherine, Leary, Sarah E S, Goldman, Stewart, Bartels, Ute, Hawkins, Cynthia, Jones, Blaise V
• Format: Journal Article
• Language: English
• Published: US Oxford University Press 26-11-2020
• Subjects: Basic and Translational Investigations; Brain Stem Neoplasms - diagnostic imaging; Brain Stem Neoplasms - genetics; Diffuse Intrinsic Pontine Glioma; Humans; Magnetic Resonance Imaging; Prospective Studies; Registries; international DIPG registry; histone mutation; diffuse midline glioma; DIPG; MRI
• ISSN: 1522-8517; 1523-5866
• DOI: 10.1093/neuonc/noaa140

Abtract
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.