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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. Greiner
  • Stewart 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
  • Cincinnati Children's Hospital Medical Center
  • Nationwide Children’s Hospital
  • Ohio State University
  • SUNY Upstate Medical University
  • Dana-Farber Cancer Institute
  • University of Toronto
  • University of British Columbia
  • Johns Hopkins University
  • Riley Hospital for Children
  • Perth Children's Hospital
  • Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia
  • The Children's Hospital, Aurora
  • Cairo University
  • Dayton Children's Hospital
  • Stanford University
  • Pediatric Hospital R. Gutierrez
  • Pennsylvania State University
  • University of Arizona
  • University of Florida
  • University of Melbourne
  • Children’s Health Queensland
  • Children's National Medical Center
  • University of Michigan, Ann Arbor
  • Seattle Children's
  • Shanghai Jiao Tong University
  • The Children's Hospital of Philadelphia
  • University of Kentucky
  • University of New South Wales
  • University of Montreal
  • St. Jude Children Research Hospital
  • Auckland District Health Board
  • University of Cincinnati

Research output: Contribution to journalArticlepeer-review

15 Scopus citations

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
StatePublished - 1 May 2022

Keywords

  • DIPG
  • central imaging review
  • histopathology
  • non-DIPG
  • pontine tumor

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