{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T08:20:09Z","timestamp":1790497209361,"version":"4.1.0"},"reference-count":39,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,2,12]],"date-time":"2021-02-12T00:00:00Z","timestamp":1613088000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["19K16579"],"award-info":[{"award-number":["19K16579"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001700","name":"Ministry of Education, Culture, Sports, Science and Technology","doi-asserted-by":"publisher","award":["JPMXS0420600120"],"award-info":[{"award-number":["JPMXS0420600120"]}],"id":[{"id":"10.13039\/501100001700","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Brain Pathology"],"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Recurrent fusion genes involving <jats:italic>C11orf95<\/jats:italic>, <jats:italic>C11orf95<\/jats:italic>\u2010<jats:italic>RELA<\/jats:italic>, have been identified only in supratentorial ependymomas among primary CNS tumors. Here, we report hitherto histopathologically unclassifiable high\u2010grade tumors, under the tentative label of \u201cependymoma\u2010like tumors with mesenchymal differentiation (ELTMDs),\u201d harboring <jats:italic>C11orf95<\/jats:italic>\u2010<jats:italic>NCOA1<\/jats:italic>\/<jats:italic>2<\/jats:italic> or \u2010<jats:italic>RELA<\/jats:italic> fusion. We examined the clinicopathological and molecular features in five cases of ELTMDs. Except for one adult case (50\u00a0years old), all cases were in children ranging from 1 to 2.5\u00a0years old. All patients presented with a mass lesion in the cerebral hemisphere. Histologically, all cases demonstrated a similar histology with a mixture of components. The major components were embryonal\u2010appearing components forming well\u2010delineated tumor cell nests composed of small uniform cells with high proliferative activity, and spindle\u2010cell mesenchymal components with a low\u2010 to high\u2010grade sarcoma\u2010like appearance. The embryonal\u2010appearing components exhibited minimal ependymal differentiation including a characteristic EMA positivity and tubular structures, but histologically did not fit with ependymoma because they lacked perivascular pseudorosettes, a histological hallmark of ependymoma, formed well\u2010delineated nests, and had diffuse and strong staining for CAM5.2. Molecular analysis identified <jats:italic>C11orf95<\/jats:italic>\u2010<jats:italic>NCOA1<\/jats:italic>, \u2010<jats:italic>NCOA2<\/jats:italic>, and \u2010<jats:italic>RELA<\/jats:italic> in two, one, and two cases, respectively. t\u2010distributed stochastic neighbor embedding analysis of DNA methylation data from two cases with <jats:italic>C11orf95<\/jats:italic>\u2010<jats:italic>NCOA1<\/jats:italic> or \u2010<jats:italic>NCOA2<\/jats:italic> and a reference set of 380 CNS tumors revealed that these two cases were clustered together and were distinct from all subgroups of ependymomas. In conclusion, although ELTMDs exhibited morphological and genetic associations with supratentorial ependymoma with <jats:italic>C11orf95<\/jats:italic>\u2010<jats:italic>RELA<\/jats:italic>, they cannot be regarded as ependymoma. Further analyses of more cases are needed to clarify their differences and similarities.<\/jats:p>","DOI":"10.1111\/bpa.12943","type":"journal-article","created":{"date-parts":[[2021,2,14]],"date-time":"2021-02-14T05:02:21Z","timestamp":1613278941000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["Ependymoma\u2010like tumor with mesenchymal differentiation harboring <i>C11orf95<\/i>\u2010<i>NCOA1<\/i>\/<i>2<\/i> or \u2010<i>RELA<\/i> fusion: A hitherto unclassified tumor related to ependymoma"],"prefix":"10.1111","volume":"31","author":[{"given":"Ran","family":"Tomomasa","sequence":"first","affiliation":[{"name":"Department of Human Pathology Gunma University Graduate School of Medicine Maebashi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuhito","family":"Arai","sequence":"additional","affiliation":[{"name":"Division of Cancer Genomics National Cancer Center Research Institute Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3504-1393","authenticated-orcid":false,"given":"Reika","family":"Kawabata\u2010Iwakawa","sequence":"additional","affiliation":[{"name":"Division of Integrated Oncology Research Gunma University Initiative for Advanced Research Maebashi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2568-0877","authenticated-orcid":false,"given":"Kohei","family":"Fukuoka","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology Saitama Children's Medical Center Saitama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshiko","family":"Nakano","sequence":"additional","affiliation":[{"name":"Division of Brain Tumor Translational Research National Cancer Center Research Institute Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natsuko","family":"Hama","sequence":"additional","affiliation":[{"name":"Division of Cancer Genomics National Cancer Center Research Institute Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Nakata","sequence":"additional","affiliation":[{"name":"Department of Oncology Johns Hopkins University School of Medicine Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nozomi","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Kitami Red Cross Hospital Kitami Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukitomo","family":"Ishi","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Faculty of Medicine Hokkaido University Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinya","family":"Tanaka","sequence":"additional","affiliation":[{"name":"Department of Cancer Pathology Faculty of Medicine, and WPI\u2010ICReDD Hokkaido University Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun A.","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Rehabilitation Medicine Rakusai Shimizu Hospital Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshiaki","family":"Yuba","sequence":"additional","affiliation":[{"name":"Department of Pathology Kitano Hospital the Tazuke Kofukai Medical Research Institute Osaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mitsutaka","family":"Shiota","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Kitano Hospital the Tazuke Kofukai Medical Research Institute Osaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsushi","family":"Natsume","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Nagoya University School of Medicine Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michihiro","family":"Kurimoto","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Nagoya University School of Medicine Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshiki","family":"Shiba","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Nagoya University School of Medicine Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikiko","family":"Aoki","sequence":"additional","affiliation":[{"name":"Department of Pathology Faculty of Medicine Fukuoka University Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuki","family":"Nabeshima","sequence":"additional","affiliation":[{"name":"Department of Pathology Faculty of Medicine Fukuoka University Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshiyuki","family":"Enomoto","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Faculty of Medicine Fukuoka University Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tooru","family":"Inoue","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Faculty of Medicine Fukuoka University Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junya","family":"Fujimura","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Adolescent Medicine Juntendo University School of Medicine Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akihide","family":"Kondo","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Juntendo University School of Medicine Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Yao","sequence":"additional","affiliation":[{"name":"Department of Human Pathology Juntendo University School of Medicine Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoki","family":"Okura","sequence":"additional","affiliation":[{"name":"Department of Radiology School of Medicine International University of Health and Welfare Narita Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7494-3496","authenticated-orcid":false,"given":"Takanori","family":"Hirose","sequence":"additional","affiliation":[{"name":"Pathology for Regional Communication Kobe University School of Medicine Kobe Japan"},{"name":"Department of Diagnostic Pathology Hyogo Cancer Center Akashi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsushi","family":"Sasaki","sequence":"additional","affiliation":[{"name":"Department of Pathology Saitama Medical University School of Medicine Moroyama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahiko","family":"Nishiyama","sequence":"additional","affiliation":[{"name":"Higashi Sapporo Hospital Sapporo Japan"},{"name":"Gunma University Maebashi Gunma Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3851-2349","authenticated-orcid":false,"given":"Koichi","family":"Ichimura","sequence":"additional","affiliation":[{"name":"Division of Brain Tumor Translational Research National Cancer Center Research Institute Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsuhiro","family":"Shibata","sequence":"additional","affiliation":[{"name":"Division of Cancer Genomics National Cancer Center Research Institute Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junko","family":"Hirato","sequence":"additional","affiliation":[{"name":"Department of Human Pathology Gunma University Graduate School of Medicine Maebashi Japan"},{"name":"Department of Pathology Public Tomioka General Hospital Tomioka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hideaki","family":"Yokoo","sequence":"additional","affiliation":[{"name":"Department of Human Pathology Gunma University Graduate School of Medicine Maebashi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9434-8400","authenticated-orcid":false,"given":"Sumihito","family":"Nobusawa","sequence":"additional","affiliation":[{"name":"Department of Human Pathology Gunma University Graduate School of Medicine Maebashi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,2,12]]},"reference":[{"key":"e_1_2_9_2_1","first-page":"106","volume-title":"World Health Organization classification of tumours of the central nervous system. Revised","author":"Ellison DW","year":"2016"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature13108"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccell.2015.04.002"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature13109"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-014-1264-4"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-012-0941-4"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccr.2011.07.007"},{"key":"e_1_2_9_9_1","first-page":"112","volume-title":"World Health Organization classification of tumours of the central nervous system. Revised","author":"Ellison DW","year":"2016"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/305427a0"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000979"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001342"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10014-020-00388-6"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.12100"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.20788"},{"key":"e_1_2_9_16_1","first-page":"621","article-title":"Localization of S\u2010100 protein and glial fibrillary acidic protein\u2010related antigen in pleomorphic adenoma of the salivary glands","volume":"46","author":"Nakazato Y","year":"1982","journal-title":"Lab Invest"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)63730-3"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1750-3639.2012.00574.x"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/nlv017"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.20731"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1097\/NEN.0b013e31816543a6"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.3500"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-018-02826-8"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12565"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-018-04622-w"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2016.01.015"},{"key":"e_1_2_9_27_1","first-page":"145","volume-title":"AFIP atlas of tumor pathology fourth series fascicle 7, tumors of the central nervous system","author":"Burger PC","year":"2007"},{"key":"e_1_2_9_28_1","first-page":"113","volume-title":"World Health Organization classification of tumours of the central nervous system. Revised","author":"Ellison DW","year":"2016"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1097\/00129039-200003000-00004"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-019-0329-2"},{"key":"e_1_2_9_31_1","first-page":"48","volume-title":"World Health Organization Classification of tumours of the central nervous system. Revised","author":"Burger PC","year":"2016"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e318158234e"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10014-014-0205-1"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22762"},{"key":"e_1_2_9_35_1","doi-asserted-by":"publisher","DOI":"10.1111\/cup.13580"},{"key":"e_1_2_9_36_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-020-02238-3"},{"key":"e_1_2_9_37_1","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/now025"},{"key":"e_1_2_9_38_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11060-018-2767-y"},{"key":"e_1_2_9_39_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10014-019-00338-x"},{"key":"e_1_2_9_40_1","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12659"}],"container-title":["Brain Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bpa.12943","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/bpa.12943","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bpa.12943","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,29]],"date-time":"2023-08-29T22:01:42Z","timestamp":1693346502000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/bpa.12943"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,2,12]]},"references-count":39,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2021,5]]}},"alternative-id":["10.1111\/bpa.12943"],"URL":"https:\/\/doi.org\/10.1111\/bpa.12943","archive":["Portico"],"relation":{},"ISSN":["1015-6305","1750-3639"],"issn-type":[{"value":"1015-6305","type":"print"},{"value":"1750-3639","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,2,12]]},"assertion":[{"value":"2020-10-12","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-01-18","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-02-12","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e12943"}}