Journal
NEURO-ONCOLOGY
Volume 22, Issue 11, Pages 1647-1657Publisher
OXFORD UNIV PRESS INC
DOI: 10.1093/neuonc/noaa140
Keywords
diffuse midline glioma; DIPG; histone mutation; international DIPG registry; MRI
Categories
Funding
- Cure Starts Now Foundation
- Julian Boivin Courage for Cures Foundation
- Hope for Caroline Foundation
- Cure Starts Now Australia
- Aidan's Avengers
- Austin Strong
- Brooke Healey Foundation
- ChadTough Foundation
- Cure Brain Cancer
- Grant's Ginormous Gift
- Jeffrey Thomas Hayden Foundation
- Love Chloe Foundation
- Musella Foundation
- Operation Grace White
- Pray Hope Believe Foundation
- Reflections of Grace
- Ryan's Hope
- Storm the Heavens Fund
- Benny's World
- Julia Barbara Foundation
- Lauren's Fight for Cure
- Lily Larue Foundation
- Isabella and Marcus Foundation
- Canadian Children's Brain Cancer Foundation
- Danni Kemp Cancer Support Fund
- Smile for Brooklyn
- Gold Hope Project
- Michael Mosier Defeat DIPG Foundation
- Soar With Grace Foundation
- Jones Family Foundation
- Smiles for Sophie Foundation
- Cure from Caleb Society
- Wayland Villars DIPG Foundation
- American Childhood Cancer Organization
- Juliana Rose Donnelly Trust
- Sheila Jones Friends
- Ellie Kavalieros DIPG Research Fund
- Voices Against Brain Cancer
- DIPG Collaborative
- Kyler Strong Foundation
- Keris Kares
- Child Health Research Institute, Stanford University
- Abbie's Army
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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.
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