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ArticleBrain tumor pathology2026

Ependymoma-like tumor with mesenchymal differentiation harboring ZFTA::NCOA2 fusion: a case report.

Masanobu Kumon, Masahiro Joko, Shigeo Ohba, Eiji Fujiwara, Makito Tanaka, Seiji Yamada, Yasuhide Takeuchi, Yohei Inoue, Akira Miyahira, Sumihito Nobusawa and 7 more

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Article in Brain tumor pathology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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2 · The registry

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4 · The record

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5 · Who and what money

Authors and funding

17 authors.

Masanobu KumonDepartment of Neurosurgery, School of Medicine, Fujita Health University, 1-98 Dengakugakubo Kutsukake-cho, Toyoake, Aichi, 470-1192, Japan.
Masahiro JokoDepartment of Neurosurgery, School of Medicine, Fujita Health University, 1-98 Dengakugakubo Kutsukake-cho, Toyoake, Aichi, 470-1192, Japan.
Shigeo OhbaDepartment of Neurosurgery, School of Medicine, Fujita Health University, 1-98 Dengakugakubo Kutsukake-cho, Toyoake, Aichi, 470-1192, Japan. shigeo.ohba@gmail.com.ORCID http://orcid.org/0000-0003-4575-0238
Eiji FujiwaraDepartment of Neurosurgery, School of Medicine, Fujita Health University, 1-98 Dengakugakubo Kutsukake-cho, Toyoake, Aichi, 470-1192, Japan.
Makito TanakaDepartment of Pediatrics, School of Medicine, Fujita Health University, Toyoake, Japan.
Seiji YamadaOncology Innovation Center, Research Promotion Headquarters, Fujita Health University School of Medicine, Toyoake, Japan.
Yasuhide TakeuchiDepartment of Diagnostic Pathology, Kyoto University Hospital, Kyoto, Japan.
Yohei InoueDepartment of Pathology, Kyorin University School of Medicine, Mitaka, Japan.
Akira MiyahiraDepartment of Pathology, Kyorin University School of Medicine, Mitaka, Japan.
Sumihito NobusawaDepartment of Human Pathology, Gunma University Graduate School of Medicine, Maebashi, Japan.
Takako YoshiokaDepartment of Pathology, National Center for Child Health and Development, Tokyo, Japan.
Junko HiratoDepartment of Pathology, Public Tomioka General Hospital, Tomioka, Japan.
Ryuta SaitoDepartment of Neurosurgery, Graduate School of Medicine, Nagoya University, Nagoya, Japan.
Akihide KondoDepartment of Neurosurgery, Juntendo University Faculty of Medicine, Tokyo, Japan.
Tetsushi YoshikawaDepartment of Pediatrics, School of Medicine, Fujita Health University, Toyoake, Japan.
Koichi IchimuraDepartment of Pathology, Kyorin University School of Medicine, Mitaka, Japan.
Yuichi HiroseDepartment of Neurosurgery, School of Medicine, Fujita Health University, 1-98 Dengakugakubo Kutsukake-cho, Toyoake, Aichi, 470-1192, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Ependymoma-like tumor with mesenchymal differentiation (ELTMD) is a recently proposed, but not yet formally defined, entity that is not recognized in the 2021 World Health Organization (WHO) classification of central nervous system tumors. Although it shares molecular features, such as ZFTA fusion, with ependymomas, it exhibits distinct histopathological and epigenetic profiles. Herein, we report the case of a 5-year-old girl with a supratentorial tumor harboring the ZFTA::NCOA2 fusion. Histopathology revealed atypical features including mesenchymal and undifferentiated components, which overlapped with those of ependymomas. DNA methylation profiling using two independent classifiers (DKFZ and NIH) yielded no matches, indicating that the tumor did not belong to any recognized CNS class. The failure of subclass assignment by both classifiers suggests that ELTMD represents a potentially epigenetically distinct subgroup. Despite being unclassifiable by the current WHO criteria, the tumor shared features with previously reported ELTMDs, supporting its recognition as an emerging tumor. This highlights the need for additional cases to refine the diagnosis, classification, and future therapeutic strategies.

Indexed as

Central nervous tumorDNA methylation profilingEpendymoma-like tumor with mesenchymal differentiationWHO classificationZFTA::NCOA2 fusion

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.