Accuracy of central neuro-imaging review of DIPG compared with histopathology in the International DIPG Registry

Margot A Lazow, Christine Fuller, Mariko DeWire, Adam Lane, Pratiti Bandopadhayay, Ute Bartels, Eric Bouffet, Sylvia Cheng, Kenneth J Cohen, Tabitha M Cooney, Scott L Coven, Hetal Dholaria, Blanca Diez, Kathleen Dorris, Moatasem El-Ayadi, Ayman El-Sheikh, Paul G Fisher, Adriana Fonseca, Mercedes Garcia Lombardi, Robert J GreinerStewart Goldman, Nicholas Gottardo, Sridharan Gururangan, Jordan R Hansford, Tim Hassall, Cynthia Hawkins, Lindsay Kilburn, Carl Koschmann, Sarah E Leary, Jie Ma, Jane E Minturn, Michelle Monje-Deisseroth, Roger Packer, Yvan Samson, Eric S Sandler, Gustavo Sevlever, Christopher L Tinkle, Karen Tsui, Lars M Wagner, Mohamed Zaghloul, David S Ziegler, Brooklyn Chaney, Katie Black, Anthony Asher, Rachid Drissi, Maryam Fouladi, Blaise V Jones, James L Leach

Research output: Contribution to journalArticlepeer-review

11 Citations (Scopus)

Abstract

BACKGROUND: Diffuse intrinsic pontine glioma (DIPG) remains a clinico-radiologic diagnosis without routine tissue acquisition. Reliable imaging distinction between DIPG and other pontine tumors with potentially more favorable prognoses and treatment considerations is essential.

METHODS: Cases submitted to the International DIPG registry (IDIPGR) with histopathologic and/or radiologic data were analyzed. Central imaging review was performed on diagnostic brain MRIs (if available) by two neuro-radiologists. Imaging features suggestive of alternative diagnoses included nonpontine origin, <50% pontine involvement, focally exophytic morphology, sharply defined margins, and/or marked diffusion restriction throughout.

RESULTS: Among 286 patients with pathology from biopsy and/or autopsy, 23 (8%) had histologic diagnoses inconsistent with DIPG, most commonly nondiffuse low-grade gliomas and embryonal tumors. Among 569 patients with centrally-reviewed diagnostic MRIs, 40 (7%) were classified as non-DIPG, alternative diagnosis suspected. The combined analysis included 151 patients with both histopathology and centrally-reviewed MRI. Of 77 patients with imaging classified as characteristic of DIPG, 76 (99%) had histopathologic diagnoses consistent with DIPG (infiltrating grade II-IV gliomas). Of 57 patients classified as likely DIPG with some unusual imaging features, 55 (96%) had histopathologic diagnoses consistent with DIPG. Of 17 patients with imaging features suggestive of an alternative diagnosis, eight (47%) had histopathologic diagnoses inconsistent with DIPG (remaining patients were excluded due to nonpontine tumor origin). Association between central neuro-imaging review impression and histopathology was significant (p < 0.001), and central neuro-imaging impression was prognostic of overall survival.

CONCLUSIONS: The accuracy and important role of central neuro-imaging review in confirming the diagnosis of DIPG is demonstrated.

Original languageEnglish
Pages (from-to)821-833
Number of pages13
JournalNeuro-Oncology
Volume24
Issue number5
DOIs
Publication statusPublished - 1 May 2022
Externally publishedYes

Fingerprint

Dive into the research topics of 'Accuracy of central neuro-imaging review of DIPG compared with histopathology in the International DIPG Registry'. Together they form a unique fingerprint.

Cite this