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

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. C...

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Bibliographic Details
Published in:Brain tumor pathology 2019-04, Vol.36 (2), p.92-101
Main Authors: Sasaki, Atsushi, Hirato, Junko, Hirose, Takanori, Fukuoka, Kohei, Kanemura, Yonehiro, Hashimoto, Naohito, Kodama, Yoshinori, Ichimura, Koichi, Sakamoto, Hiroaki, Nishikawa, Ryo
Format: Article
Language:English
Subjects:
Antibodies
Automation
Brain cancer
Brain research
Cancer Research
Medical prognosis
Medical research
Medicine
Medicine & Public Health
Neurology
Neurosurgery
Oncology
Original Article
Pathology
Pediatrics
Spinal cord
Stains & staining
Tumors
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Summary: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.
ISSN:1433-7398
1861-387X
DOI:10.1007/s10014-019-00338-x