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