Accuracy of central neuro-imaging review of DIPG compared with histopathology in the International DIPG Registry. Issue 5 (20th October 2021)

• Record Type: Journal Article
• Authors: Lazow, Margot A; Fuller, Christine; DeWire, Mariko; Lane, Adam; Bandopadhayay, Pratiti; Bartels, Ute; Bouffet, Eric; Cheng, Sylvia; Cohen, Kenneth J; Cooney, Tabitha M; Coven, Scott L; Dholaria, Hetal; Diez, Blanca; Dorris, Kathleen; El-ayadi, Moatasem; El-Sheikh, Ayman; Fisher, Paul G; Fonseca, Adriana; Garcia Lombardi, Mercedes; Greiner, Robert J; Goldman, Stewart; Gottardo, Nicholas; Gururangan, Sridharan; Hansford, Jordan R; Hassall, Tim; Hawkins, Cynthia; Kilburn, Lindsay; Koschmann, Carl; Leary, Sarah E; Ma, Jie; Minturn, Jane E; Monje-Deisseroth, Michelle; Packer, Roger; Samson, Yvan; Sandler, Eric S; Sevlever, Gustavo; Tinkle, Christopher L; Tsui, Karen; Wagner, Lars M; Zaghloul, Mohamed; Ziegler, David S; Chaney, Brooklyn; Black, Katie; Asher, Anthony; Drissi, Rachid; Fouladi, Maryam; Jones, Blaise V; Leach, James L;
• Is Part Of: Neuro-oncology. Volume 24:Issue 5(2022)
• Page Start: 821
• Page End: 833
• Publication Date: 2021-10-20
• Subjects: central imaging review -- DIPG -- histopathology -- non-DIPG -- pontine tumor; Brain Neoplasms -- Periodicals; Brain -- Tumors -- Periodicals; Brain -- Cancer -- Periodicals; Nervous system -- Cancer -- Periodicals; 616.99481
• DOI: 10.1093/neuonc/noab245
• Languages: English
• ISSNs: 1522-8517

Abstract

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.