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Published in: Brain Tumor Pathology 2/2019

01-04-2019 | Ependymoma | Original Article

Review of ependymomas: assessment of consensus in pathological diagnosis and correlations with genetic profiles and outcome

Authors: Atsushi Sasaki, Junko Hirato, Takanori Hirose, Kohei Fukuoka, Yonehiro Kanemura, Naohito Hashimoto, Yoshinori Kodama, Koichi Ichimura, Hiroaki Sakamoto, Ryo Nishikawa

Published in: Brain Tumor Pathology | Issue 2/2019

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Abstract

We focused on histological and immunohistochemical characteristics of ependymoma (EPN) with molecular profiles to develop more reproducible criteria of the diagnosis. Three expert neuropathologists reviewed the pathology of 130 samples from the Japan Pediatric Molecular Neuro-Oncology Group study. Confirmed cases were assessed for histology, surrogate markers, molecular subgrouping, and survival data. We reached a consensus regarding the diagnosis of EPNs in 100% of spinal cord tumors and 93% of posterior fossa (PF) tumors that had been diagnosed as EPNs by local pathologists, whereas we reached a consensus regarding only 77% of the local diagnosis of supratentorial (ST) EPNs. Among the PF-EPNs, most of anaplastic ependymomas (AEPNs) were defined as EPN-A by methylation profiling, which was significantly correlated with the subgroup assignment. Regarding prognosis, the overall survival of patients with PF-EPN was significantly better than that of patients with PF AEPN (p = 0.01). Histologically, all ependymoma, RELA fusion-positive (EPN-RELA) qualified as Grade III. Both L1 cell adhesion molecule and nuclear factor kappaB p65 antibodies showed good sensitivity for detecting EPN-RELA. This study indicated that the expert consensus pathological diagnosis could correlate well with the molecular classifications in EPNs. ST EPNs should be diagnosed more carefully by histological and molecular analyses.
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Metadata
Title
Review of ependymomas: assessment of consensus in pathological diagnosis and correlations with genetic profiles and outcome
Authors
Atsushi Sasaki
Junko Hirato
Takanori Hirose
Kohei Fukuoka
Yonehiro Kanemura
Naohito Hashimoto
Yoshinori Kodama
Koichi Ichimura
Hiroaki Sakamoto
Ryo Nishikawa
Publication date
01-04-2019
Publisher
Springer Singapore
Published in
Brain Tumor Pathology / Issue 2/2019
Print ISSN: 1433-7398
Electronic ISSN: 1861-387X
DOI
https://doi.org/10.1007/s10014-019-00338-x

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Acknowledgment

Reviewers in 2018

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Preface