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