TY - JOUR
T1 - MR imaging features of diffuse intrinsic pontine glioma and relationship to overall survival
T2 - Report from the International DIPG Registry
AU - Leach, James L.
AU - Roebker, James
AU - Schafer, Austin
AU - Baugh, Joshua
AU - Chaney, Brooklyn
AU - Fuller, Christine
AU - Fouladi, Maryam
AU - Lane, Adam
AU - Doughman, Renee
AU - Drissi, Rachid
AU - DeWire-Schottmiller, Mariko
AU - Ziegler, David S.
AU - Minturn, Jane E.
AU - Hansford, Jordan R.
AU - Wang, Stacie S.
AU - Monje-Deisseroth, Michelle
AU - Fisher, Paul G.
AU - Gottardo, Nicholas G.
AU - Dholaria, Hetal
AU - Packer, Roger
AU - Warren, Katherine
AU - Leary, Sarah E.S.
AU - Goldman, Stewart
AU - Bartels, Ute
AU - Hawkins, Cynthia
AU - Jones, Blaise V.
N1 - Publisher Copyright:
© The Author(s) 2020. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved.
PY - 2020/11/1
Y1 - 2020/11/1
N2 - 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.
AB - 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.
KW - Diffuse midline glioma
KW - DIPG
KW - Histone mutation
KW - International DIPG registry
KW - MRI
UR - http://www.scopus.com/inward/record.url?scp=85087650565&partnerID=8YFLogxK
U2 - 10.1093/neuonc/noaa140
DO - 10.1093/neuonc/noaa140
M3 - Article
C2 - 32506137
AN - SCOPUS:85087650565
SN - 1522-8517
VL - 22
SP - 1647
EP - 1657
JO - Neuro-Oncology
JF - Neuro-Oncology
IS - 11
ER -