{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T08:20:06Z","timestamp":1790497206549,"version":"4.1.0"},"reference-count":28,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,8,13]],"date-time":"2021-08-13T00:00:00Z","timestamp":1628812800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,8,13]],"date-time":"2021-08-13T00:00:00Z","timestamp":1628812800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["acta neuropathol commun"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The cIMPACT-NOW Update 7 has replaced the WHO nosology of \u201cependymoma, <jats:italic>RELA<\/jats:italic> fusion positive\u201d by \u201cSupratentorial-ependymoma, <jats:italic>C11orf95<\/jats:italic>-fusion positive\u201d. This modification reinforces the idea that supratentorial-ependymomas exhibiting fusion that implicates the <jats:italic>C11orf95<\/jats:italic> (now called <jats:italic>ZFTA<\/jats:italic>) gene with or without the <jats:italic>RELA<\/jats:italic> gene, represent the same histomolecular entity. A hot off the press molecular study has identified distinct clusters of the DNA methylation class of <jats:italic>ZFTA<\/jats:italic> fusion-positive tumors. Interestingly, clusters 2 and 4 comprised tumors of different morphologies, with various <jats:italic>ZFTA<\/jats:italic> fusions without involvement of <jats:italic>RELA.<\/jats:italic> In this paper, we present a detailed series of thirteen cases of non-<jats:italic>RELA ZFTA<\/jats:italic>-fused supratentorial tumors with extensive clinical, radiological, histopathological, immunohistochemical, genetic and epigenetic (DNA methylation profiling) characterization. Contrary to the age of onset and MRI aspects similar to <jats:italic>RELA<\/jats:italic> fusion-positive EPN, we noted significant histopathological heterogeneity (pleomorphic xanthoastrocytoma-like, astroblastoma-like, ependymoma-like, and even sarcoma-like patterns) in this cohort. Immunophenotypically, these NF\u03baB immunonegative tumors expressed GFAP variably, but EMA constantly and L1CAM frequently. Different gene partners were fused with <jats:italic>ZFTA<\/jats:italic>: <jats:italic>NCOA1\/2<\/jats:italic>, <jats:italic>MAML2<\/jats:italic> and for the first time <jats:italic>MN1<\/jats:italic>. These tumors had epigenetic homologies within the DNA methylation class of ependymomas-RELA and were classified as satellite clusters 2 and 4. Cluster 2 (n\u2009=\u20099) corresponded to tumors with classic ependymal histological features (n\u2009=\u20094) but also had astroblastic features (n\u2009=\u20095). Various types of <jats:italic>ZFTA<\/jats:italic> fusions were associated with cluster 2, but as in the original report, <jats:italic>ZFTA:MAML2<\/jats:italic> fusion was frequent. Cluster 4 was enriched with sarcoma-like tumors. Moreover, we reported a novel anatomy of three <jats:italic>ZFTA:NCOA1\/2<\/jats:italic> fusions with only 1 ZFTA zinc finger domain in the putative fusion protein, whereas all previously reported non-<jats:italic>RELA ZFTA<\/jats:italic> fusions have 4 ZFTA zinc fingers. All three cases presented a sarcoma-like morphology. This genotype\/phenotype association requires further studies for confirmation. Our series is the first to extensively characterize this new subset of supratentorial <jats:italic>ZFTA<\/jats:italic>-fused ependymomas and highlights the usefulness of <jats:italic>ZFTA<\/jats:italic> FISH analysis to confirm the existence of a rearrangement without <jats:italic>RELA<\/jats:italic> abnormality.<\/jats:p>","DOI":"10.1186\/s40478-021-01238-y","type":"journal-article","created":{"date-parts":[[2021,8,13]],"date-time":"2021-08-13T04:03:45Z","timestamp":1628827425000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":67,"title":["Supratentorial non-RELA, ZFTA-fused ependymomas: a comprehensive phenotype genotype correlation highlighting the number of zinc fingers in ZFTA-NCOA1\/2 fusions"],"prefix":"10.1186","volume":"9","author":[{"given":"Arnault","family":"Tauzi\u00e8de-Espariat","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aurore","family":"Siegfried","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yvan","family":"Nicaise","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Kergrohen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philipp","family":"Sievers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre","family":"Vasiljevic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre","family":"Roux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edouard","family":"Dezamis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Benevello","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie-Christine","family":"Machet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Michalak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chloe","family":"Puiseux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francisco","family":"Llamas-Gutierrez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Leblond","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franck","family":"Bourdeaut","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacques","family":"Grill","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christelle","family":"Dufour","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L\u00e9a","family":"Guerrini-Rousseau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuel","family":"Abbou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Volodia","family":"Dangouloff-Ros","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Boddaert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rapha\u00ebl","family":"Saffroy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren","family":"Hasty","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen","family":"Wahler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00e9lanie","family":"Pag\u00e8s","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felipe","family":"Andreiuolo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanu\u00e8le","family":"Lechapt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabrice","family":"Chr\u00e9tien","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Blauwblomme","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K\u00e9vin","family":"Beccaria","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johan","family":"Pallud","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phanie","family":"Puget","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Uro-Coste","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascale","family":"Varlet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the RENOCLIP-LOC, the BIOMECA (Biomarkers for Ependymomas in Children, Adolescents) consortium","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,8,13]]},"reference":[{"key":"1238_CR1","doi-asserted-by":"publisher","first-page":"728","DOI":"10.1016\/j.ccell.2015.04.002","volume":"27","author":"KW Pajtler","year":"2015","unstructured":"Pajtler KW, Witt H, Sill M, Jones DTW, Hovestadt V, Kratochwil F, Wani K, Tatevossian R, Punchihewa C, Johann P, Reimand J, Warnatz H-J, Ryzhova M, Mack S, Ramaswamy V, Capper D, Schweizer L, Sieber L, Wittmann A, Huang Z, van Sluis P, Volckmann R, Koster J, Versteeg R, Fults D, Toledano H, Avigad S, Hoffman LM, Donson AM, Foreman N, Hewer E, Zitterbart K, Gilbert M, Armstrong TS, Gupta N, Allen JC, Karajannis MA, Zagzag D, Hasselblatt M, Kulozik AE, Witt O, Collins VP, von Hoff K, Rutkowski S, Pietsch T, Bader G, Yaspo M-L, von Deimling A, Lichter P, Taylor MD, Gilbertson R, Ellison DW, Aldape K, Korshunov A, Kool M, Pfister SM (2015) Molecular classification of ependymal tumors across all CNS compartments, histopathological grades, and age groups. Cancer Cell 27:728\u2013743. https:\/\/doi.org\/10.1016\/j.ccell.2015.04.002","journal-title":"Cancer Cell"},{"key":"1238_CR2","doi-asserted-by":"publisher","first-page":"205","DOI":"10.1111\/bpa.12659","volume":"29","author":"F Andreiuolo","year":"2019","unstructured":"Andreiuolo F, Varlet P, Tauzi\u00e8de-Espariat A, J\u00fcnger ST, D\u00f6rner E, Dreschmann V, Kuchelmeister K, Waha A, Haberler C, Slavc I, Corbacioglu S, Riemenschneider MJ, Leipold A, R\u00fcdiger T, K\u00f6rholz D, Acker T, Russo A, Faber J, Sommer C, Armbrust S, Rose M, Erdlenbruch B, Hans VH, Bernbeck B, Schneider D, Lorenzen J, Ebinger M, Handgretinger R, Neumann M, van Buiren M, Prinz M, Roganovic J, Jakovcevic A, Park S-H, Grill J, Puget S, Messing-J\u00fcnger M, Reinhard H, Bergmann M, Hattingen E, Pietsch T (2019) Childhood supratentorial ependymomas with YAP1-MAMLD1 fusion: an entity with characteristic clinical, radiological, cytogenetic and histopathological features. Brain Pathol Zurich Switz 29:205\u2013216. https:\/\/doi.org\/10.1111\/bpa.12659","journal-title":"Brain Pathol Zurich Switz"},{"key":"1238_CR3","doi-asserted-by":"publisher","first-page":"325","DOI":"10.1111\/bpa.12664","volume":"29","author":"M Pag\u00e8s","year":"2019","unstructured":"Pag\u00e8s M, Pajtler KW, Puget S, Castel D, Boddaert N, Tauzi\u00e8de-Espariat A, Picot S, Debily M-A, Kool M, Capper D, Sainte-Rose C, Chr\u00e9tien F, Pfister SM, Pietsch T, Grill J, Varlet P, Andreiuolo F (2019) Diagnostics of pediatric supratentorial RELA ependymomas: integration of information from histopathology, genetics, DNA methylation and imaging. Brain Pathol Zurich Switz 29:325\u2013335. https:\/\/doi.org\/10.1111\/bpa.12664","journal-title":"Brain Pathol Zurich Switz"},{"key":"1238_CR4","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1038\/nature13109","volume":"506","author":"M Parker","year":"2014","unstructured":"Parker M, Mohankumar KM, Punchihewa C, Weinlich R, Dalton JD, Li Y, Lee R, Tatevossian RG, Phoenix TN, Thiruvenkatam R, White E, Tang B, Orisme W, Gupta K, Rusch M, Chen X, Li Y, Nagahawhatte P, Hedlund E, Finkelstein D, Wu G, Shurtleff S, Easton J, Boggs K, Yergeau D, Vadodaria B, Mulder HL, Becksfort J, Becksford J, Gupta P, Huether R, Ma J, Song G, Gajjar A, Merchant T, Boop F, Smith AA, Ding L, Lu C, Ochoa K, Zhao D, Fulton RS, Fulton LL, Mardis ER, Wilson RK, Downing JR, Green DR, Zhang J, Ellison DW, Gilbertson RJ (2014) C11orf95-RELA fusions drive oncogenic NF-\u03baB signalling in ependymoma. Nature 506:451\u2013455. https:\/\/doi.org\/10.1038\/nature13109","journal-title":"Nature"},{"key":"1238_CR5","doi-asserted-by":"publisher","first-page":"863","DOI":"10.1111\/bpa.12866","volume":"30","author":"DW Ellison","year":"2020","unstructured":"Ellison DW, Aldape KD, Capper D, Fouladi M, Gilbert MR, Gilbertson RJ, Hawkins C, Merchant TE, Pajtler K, Venneti S, Louis DN (2020) cIMPACT-NOW update 7: advancing the molecular classification of ependymal tumors. Brain Pathol Zurich Switz 30:863\u2013866. https:\/\/doi.org\/10.1111\/bpa.12866","journal-title":"Brain Pathol Zurich Switz"},{"key":"1238_CR6","doi-asserted-by":"publisher","first-page":"64","DOI":"10.1007\/s10014-020-00388-6","volume":"38","author":"S Tamai","year":"2021","unstructured":"Tamai S, Nakano Y, Kinoshita M, Sabit H, Nobusawa S, Arai Y, Hama N, Totoki Y, Shibata T, Ichimura K, Nakada M (2021) Ependymoma with C11orf95-MAML2 fusion: presenting with granular cell and ganglion cell features. Brain Tumor Pathol 38:64\u201370. https:\/\/doi.org\/10.1007\/s10014-020-00388-6","journal-title":"Brain Tumor Pathol"},{"key":"1238_CR7","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12943","author":"R Tomomasa","year":"2021","unstructured":"Tomomasa R, Arai Y, Kawabata-Iwakawa R, Fukuoka K, Nakano Y, Hama N, Nakata S, Suzuki N, Ishi Y, Tanaka S, Takahashi JA, Yuba Y, Shiota M, Natsume A, Kurimoto M, Shiba Y, Aoki M, Nabeshima K, Enomoto T, Inoue T, Fujimura J, Kondo A, Yao T, Okura N, Hirose T, Sasaki A, Nishiyama M, Ichimura K, Shibata T, Hirato J, Yokoo H, Nobusawa S (2021) Ependymoma-like tumor with mesenchymal differentiation harboring C11orf95-NCOA1\/2 or -RELA fusion: a hitherto unclassified tumor related to ependymoma. Brain Pathol Zurich Switz. https:\/\/doi.org\/10.1111\/bpa.12943","journal-title":"Brain Pathol Zurich Switz"},{"key":"1238_CR8","doi-asserted-by":"publisher","first-page":"455","DOI":"10.1007\/s00401-020-02260-5","volume":"141","author":"V Zschernack","year":"2021","unstructured":"Zschernack V, J\u00fcnger ST, Mynarek M, Rutkowski S, Garre ML, Ebinger M, Neu M, Faber J, Erdlenbruch B, Claviez A, Bielack S, Brozou T, Fr\u00fchwald MC, D\u00f6rner E, Dreschmann V, Stock A, Solymosi L, Hench J, Frank S, Vokuhl C, Waha A, Andreiuolo F, Pietsch T (2021) Supratentorial ependymoma in childhood: more than just RELA or YAP. Acta Neuropathol (Berl) 141:455\u2013466. https:\/\/doi.org\/10.1007\/s00401-020-02260-5","journal-title":"Acta Neuropathol (Berl)"},{"key":"1238_CR9","doi-asserted-by":"publisher","DOI":"10.1158\/2159-8290.CD-20-0963","author":"T Zheng","year":"2021","unstructured":"Zheng T, Ghasemi DR, Okonechnikov K, Korshunov A, Sill M, Maass KK, Goncalves B, da Silva P, Ryzhova M, Gojo J, Stichel D, Arabzade A, Kupp R, Benzel J, Taya S, Adachi T, Shiraishi R, Gerber NU, Sturm D, Ecker J, Sievers P, Selt F, Chapman R, Haberler C, Figarella-Branger D, Reifenberger G, Fleischhack G, Rutkowski S, Donson AM, Ramaswamy V, Capper D, Ellison DW, Herold-Mende CC, Schuller U, Brandner S, Hernaiz Driever P, Kros JM, Snuderl M, Milde T, Grundy RG, Hoshino M, Mack SC, Gilbertson RJ, Jones DTW, Kool M, von Deimling A, Pfister SM, Sahm F, Kawauchi D, Pajtler KW (2021) Cross-species genomics reveals oncogenic dependencies in ZFTA\/C11orf95 fusion-positive supratentorial ependymomas. Cancer Discov. https:\/\/doi.org\/10.1158\/2159-8290.CD-20-0963","journal-title":"Cancer Discov"},{"key":"1238_CR10","doi-asserted-by":"publisher","first-page":"e153","DOI":"10.18295\/squmj.2019.19.02.012","volume":"19","author":"A Al-Battashi","year":"2019","unstructured":"Al-Battashi A, Al Hajri Z, Perry A, Al-Kindi H, Al-Ghaithi I (2019) A cerebellar high-grade neuroepithelial tumour with BCOR alteration in a five-year-old child: a case report. Sultan Qaboos Univ Med J 19:e153\u2013e156. https:\/\/doi.org\/10.18295\/squmj.2019.19.02.012","journal-title":"Sultan Qaboos Univ Med J"},{"key":"1238_CR11","doi-asserted-by":"publisher","first-page":"1254","DOI":"10.1097\/PAS.0000000000000866","volume":"41","author":"R Appay","year":"2017","unstructured":"Appay R, Macagno N, Padovani L, Korshunov A, Kool M, Andr\u00e9 N, Scavarda D, Pietsch T, Figarella-Branger D (2017) HGNET-BCOR tumors of the cerebellum: clinicopathologic and molecular characterization of 3 cases. Am J Surg Pathol 41:1254\u20131260. https:\/\/doi.org\/10.1097\/PAS.0000000000000866","journal-title":"Am J Surg Pathol"},{"key":"1238_CR12","doi-asserted-by":"publisher","DOI":"10.1634\/theoncologist.2019-0223","author":"W Boisseau","year":"2019","unstructured":"Boisseau W, Euskirchen P, Mokhtari K, Dehais C, Touat M, Hoang-Xuan K, Sanson M, Capelle L, Nouet A, Karachi C, Bielle F, Gu\u00e9gan J, Marie Y, Martin-Duverneuil N, Taillandier L, Rousseau A, Delattre J-Y, Idbaih A (2019) Molecular profiling reclassifies adult astroblastoma into known and clinically distinct tumor entities with frequent mitogen-activated protein kinase pathway alterations. Oncologist. https:\/\/doi.org\/10.1634\/theoncologist.2019-0223","journal-title":"Oncologist"},{"key":"1238_CR13","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.28384","author":"J Bremer","year":"2020","unstructured":"Bremer J, Kottke R, Johann PD, von Hoff K, Brazzola P, Grotzer MA, Kool M, Rushing E, Gerber NU (2020) A single supratentorial high-grade neuroepithelial tumor with two distinct BCOR mutations, exceptionally long complete remission and survival. Pediatr Blood Cancer. https:\/\/doi.org\/10.1002\/pbc.28384","journal-title":"Pediatr Blood Cancer"},{"key":"1238_CR14","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12747","author":"SP Ferris","year":"2019","unstructured":"Ferris SP, Velazquez Vega J, Aboian M, Lee JC, Van Ziffle J, Onodera C, Grenert JP, Saunders T, Chen Y-Y, Banerjee A, Kline CN, Gupta N, Raffel C, Samuel D, Ruiz-Diaz I, Magaki S, Wilson D, Neltner J, Al-Hajri Z, Phillips JJ, Pekmezci M, Bollen AW, Tihan T, Schniederjan M, Cha S, Perry A, Solomon DA (2019) High-grade neuroepithelial tumor with BCOR exon 15 internal tandem duplication\u2014a comprehensive clinical, radiographic, pathologic, and genomic analysis. Brain Pathol Zurich Switz. https:\/\/doi.org\/10.1111\/bpa.12747","journal-title":"Brain Pathol Zurich Switz"},{"key":"1238_CR15","doi-asserted-by":"publisher","first-page":"134","DOI":"10.1186\/s40478-018-0630-1","volume":"6","author":"K Fukuoka","year":"2018","unstructured":"Fukuoka K, Kanemura Y, Shofuda T, Fukushima S, Yamashita S, Narushima D, Kato M, Honda-Kitahara M, Ichikawa H, Kohno T, Sasaki A, Hirato J, Hirose T, Komori T, Satomi K, Yoshida A, Yamasaki K, Nakano Y, Takada A, Nakamura T, Takami H, Matsushita Y, Suzuki T, Nakamura H, Makino K, Sonoda Y, Saito R, Tominaga T, Matsusaka Y, Kobayashi K, Nagane M, Furuta T, Nakada M, Narita Y, Hirose Y, Ohba S, Wada A, Shimizu K, Kurozumi K, Date I, Fukai J, Miyairi Y, Kagawa N, Kawamura A, Yoshida M, Nishida N, Wataya T, Yamaoka M, Tsuyuguchi N, Uda T, Takahashi M, Nakano Y, Akai T, Izumoto S, Nonaka M, Yoshifuji K, Kodama Y, Mano M, Ozawa T, Ramaswamy V, Taylor MD, Ushijima T, Shibui S, Yamasaki M, Arai H, Sakamoto H, Nishikawa R, Ichimura K, Japan Pediatric Molecular Neuro-Oncology Group (JPMNG) (2018) Significance of molecular classification of ependymomas: C11orf95-RELA fusion-negative supratentorial ependymomas are a heterogeneous group of tumors. Acta Neuropathol Commun 6:134. https:\/\/doi.org\/10.1186\/s40478-018-0630-1","journal-title":"Acta Neuropathol Commun"},{"key":"1238_CR16","doi-asserted-by":"publisher","first-page":"279","DOI":"10.1016\/j.wneu.2018.05.158","volume":"116","author":"MA Kirkman","year":"2018","unstructured":"Kirkman MA, Pickles JC, Fairchild AR, Avery A, Pietsch T, Jacques TS, Aquilina K (2018) Early wound site seeding in a patient with central nervous system high-grade neuroepithelial tumor with BCOR alteration. World Neurosurg 116:279\u2013284. https:\/\/doi.org\/10.1016\/j.wneu.2018.05.158","journal-title":"World Neurosurg"},{"key":"1238_CR17","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1186\/s40478-019-0689-3","volume":"7","author":"NL Lehman","year":"2019","unstructured":"Lehman NL, Usubalieva A, Lin T, Allen SJ, Tran QT, Mobley BC, McLendon RE, Schniederjan MJ, Georgescu M-M, Couce M, Dulai MS, Raisanen JM, Al Abbadi M, Palmer CA, Hattab EM, Orr BA (2019) Genomic analysis demonstrates that histologically-defined astroblastomas are molecularly heterogeneous and that tumors with MN1 rearrangement exhibit the most favorable prognosis. Acta Neuropathol Commun 7:42. https:\/\/doi.org\/10.1186\/s40478-019-0689-3","journal-title":"Acta Neuropathol Commun"},{"key":"1238_CR18","doi-asserted-by":"publisher","DOI":"10.3389\/fgene.2019.00391","author":"G Petruzzellis","year":"2019","unstructured":"Petruzzellis G, Alessi I, Colafati GS, Diomedi-Camassei F, Ciolfi A, Pedace L, Cacchione A, Carai A, Tartaglia M, Mastronuzzi A, Miele E (2019) Role of DNA methylation profile in diagnosing astroblastoma: a case report and literature review. Front Genet. https:\/\/doi.org\/10.3389\/fgene.2019.00391","journal-title":"Front Genet"},{"key":"1238_CR19","doi-asserted-by":"publisher","first-page":"1060","DOI":"10.1016\/j.cell.2016.01.015","volume":"164","author":"D Sturm","year":"2016","unstructured":"Sturm D, Orr BA, Toprak UH, Hovestadt V, Jones DTW, Capper D, Sill M, Buchhalter I, Northcott PA, Leis I, Ryzhova M, Koelsche C, Pfaff E, Allen SJ, Balasubramanian G, Worst BC, Pajtler KW, Brabetz S, Johann PD, Sahm F, Reimand J, Mackay A, Carvalho DM, Remke M, Phillips JJ, Perry A, Cowdrey C, Drissi R, Fouladi M, Giangaspero F, \u0141astowska M, Grajkowska W, Scheurlen W, Pietsch T, Hagel C, Gojo J, L\u00f6tsch D, Berger W, Slavc I, Haberler C, Jouvet A, Holm S, Hofer S, Prinz M, Keohane C, Fried I, Mawrin C, Scheie D, Mobley BC, Schniederjan MJ, Santi M, Buccoliero AM, Dahiya S, Kramm CM, von Bueren AO, von Hoff K, Rutkowski S, Herold-Mende C, Fr\u00fchwald MC, Milde T, Hasselblatt M, Wesseling P, R\u00f6\u00dfler J, Sch\u00fcller U, Ebinger M, Schittenhelm J, Frank S, Grobholz R, Vajtai I, Hans V, Schneppenheim R, Zitterbart K, Collins VP, Aronica E, Varlet P, Puget S, Dufour C, Grill J, Figarella-Branger D, Wolter M, Schuhmann MU, Shalaby T, Grotzer M, van Meter T, Monoranu C-M, Felsberg J, Reifenberger G, Snuderl M, Forrester LA, Koster J, Versteeg R, Volckmann R, van Sluis P, Wolf S, Mikkelsen T, Gajjar A, Aldape K, Moore AS, Taylor MD, Jones C, Jabado N, Karajannis MA, Eils R, Schlesner M, Lichter P, von Deimling A, Pfister SM, Ellison DW, Korshunov A, Kool M (2016) New brain tumor entities emerge from molecular classification of CNS-PNETs. Cell 164:1060\u20131072. https:\/\/doi.org\/10.1016\/j.cell.2016.01.015","journal-title":"Cell"},{"key":"1238_CR20","doi-asserted-by":"publisher","first-page":"176","DOI":"10.1186\/s40478-019-0834-z","volume":"7","author":"A Tauzi\u00e8de-Espariat","year":"2019","unstructured":"Tauzi\u00e8de-Espariat A, Pag\u00e8s M, Roux A, Siegfried A, Uro-Coste E, Nicaise Y, Sevely A, Gambart M, Boetto S, Dupuy M, Richard P, Perbet R, Vinchon M, Caron S, Andreiuolo F, Gareton A, Lechapt E, Chr\u00e9tien F, Puget S, Grill J, Boddaert N, Varlet P, RENOCLIP-LOC, (2019) Pediatric methylation class HGNET-MN1: unresolved issues with terminology and grading. Acta Neuropathol Commun 7:176. https:\/\/doi.org\/10.1186\/s40478-019-0834-z","journal-title":"Acta Neuropathol Commun"},{"key":"1238_CR21","doi-asserted-by":"publisher","first-page":"192","DOI":"10.1111\/bpa.12561","volume":"28","author":"MD Wood","year":"2018","unstructured":"Wood MD, Tihan T, Perry A, Chacko G, Turner C, Pu C, Payne C, Yu A, Bannykh SI, Solomon DA (2018) Multimodal molecular analysis of astroblastoma enables reclassification of most cases into more specific molecular entities. Brain Pathol Zurich Switz 28:192\u2013202. https:\/\/doi.org\/10.1111\/bpa.12561","journal-title":"Brain Pathol Zurich Switz"},{"key":"1238_CR22","doi-asserted-by":"publisher","first-page":"710","DOI":"10.1111\/bpa.12585","volume":"28","author":"Y Yoshida","year":"2018","unstructured":"Yoshida Y, Nobusawa S, Nakata S, Nakada M, Arakawa Y, Mineharu Y, Sugita Y, Yoshioka T, Araki A, Sato Y, Takeshima H, Okada M, Nishi A, Yamazaki T, Kohashi K, Oda Y, Hirato J, Yokoo H (2018) CNS high-grade neuroepithelial tumor with BCOR internal tandem duplication: a comparison with its counterparts in the kidney and soft tissue. Brain Pathol Zurich Switz 28:710\u2013720. https:\/\/doi.org\/10.1111\/bpa.12585","journal-title":"Brain Pathol Zurich Switz"},{"key":"1238_CR23","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12473","author":"M Pag\u00e8s","year":"2016","unstructured":"Pag\u00e8s M, Beccaria K, Boddaert N, Saffroy R, Besnard A, Castel D, Fina F, Barets D, Barret E, Lacroix L, Bielle F, Andreiuolo F, Tauzi\u00e8de-Espariat A, Figarella-Branger D, Puget S, Grill J, Chr\u00e9tien F, Varlet P (2016) Co-occurrence of histone H3 K27M and BRAF V600E mutations in paediatric midline grade I ganglioglioma. Brain Pathol Zurich Switz. https:\/\/doi.org\/10.1111\/bpa.12473","journal-title":"Brain Pathol Zurich Switz"},{"key":"1238_CR24","doi-asserted-by":"publisher","first-page":"469","DOI":"10.1038\/nature26000","volume":"555","author":"D Capper","year":"2018","unstructured":"Capper D, Jones DTW, Sill M, Hovestadt V, Schrimpf D, Sturm D, Koelsche C, Sahm F, Chavez L, Reuss DE, Kratz A, Wefers AK, Huang K, Pajtler KW, Schweizer L, Stichel D, Olar A, Engel NW, Lindenberg K, Harter PN, Braczynski AK, Plate KH, Dohmen H, Garvalov BK, Coras R, H\u00f6lsken A, Hewer E, Bewerunge-Hudler M, Schick M, Fischer R, Beschorner R, Schittenhelm J, Staszewski O, Wani K, Varlet P, Pages M, Temming P, Lohmann D, Selt F, Witt H, Milde T, Witt O, Aronica E, Giangaspero F, Rushing E, Scheurlen W, Geisenberger C, Rodriguez FJ, Becker A, Preusser M, Haberler C, Bjerkvig R, Cryan J, Farrell M, Deckert M, Hench J, Frank S, Serrano J, Kannan K, Tsirigos A, Br\u00fcck W, Hofer S, Brehmer S, Seiz-Rosenhagen M, H\u00e4nggi D, Hans V, Rozsnoki S, Hansford JR, Kohlhof P, Kristensen BW, Lechner M, Lopes B, Mawrin C, Ketter R, Kulozik A, Khatib Z, Heppner F, Koch A, Jouvet A, Keohane C, M\u00fchleisen H, Mueller W, Pohl U, Prinz M, Benner A, Zapatka M, Gottardo NG, Driever PH, Kramm CM, M\u00fcller HL, Rutkowski S, von Hoff K, Fr\u00fchwald MC, Gnekow A, Fleischhack G, Tippelt S, Calaminus G, Monoranu C-M, Perry A, Jones C, Jacques TS, Radlwimmer B, Gessi M, Pietsch T, Schramm J, Schackert G, Westphal M, Reifenberger G, Wesseling P, Weller M, Collins VP, Bl\u00fcmcke I, Bendszus M, Debus J, Huang A, Jabado N, Northcott PA, Paulus W, Gajjar A, Robinson GW, Taylor MD, Jaunmuktane Z, Ryzhova M, Platten M, Unterberg A, Wick W, Karajannis MA, Mittelbronn M, Acker T, Hartmann C, Aldape K, Sch\u00fcller U, Buslei R, Lichter P, Kool M, Herold-Mende C, Ellison DW, Hasselblatt M, Snuderl M, Brandner S, Korshunov A, von Deimling A, Pfister SM (2018) DNA methylation-based classification of central nervous system tumours. Nature 555:469\u2013474. https:\/\/doi.org\/10.1038\/nature26000","journal-title":"Nature"},{"key":"1238_CR25","doi-asserted-by":"publisher","first-page":"919","DOI":"10.1093\/neuonc\/now025","volume":"18","author":"D Figarella-Branger","year":"2016","unstructured":"Figarella-Branger D, Lechapt-Zalcman E, Tabouret E, J\u00fcnger S, de Paula AM, Bouvier C, Colin C, Jouvet A, Forest F, Andreiuolo F, Quintin-Roue I, Machet M-C, Heitzmann A, Milin S, Sevestre H, Godfraind C, Labrousse F, Metellus P, Scavarda D, Pietsch T (2016) Supratentorial clear cell ependymomas with branching capillaries demonstrate characteristic clinicopathological features and pathological activation of nuclear factor-kappaB signaling. Neuro Oncol 18:919\u2013927. https:\/\/doi.org\/10.1093\/neuonc\/now025","journal-title":"Neuro Oncol"},{"key":"1238_CR26","doi-asserted-by":"publisher","first-page":"56","DOI":"10.1097\/PAS.0000000000000979","volume":"43","author":"M Gessi","year":"2019","unstructured":"Gessi M, Giagnacovo M, Modena P, Elefante G, Gianno F, Buttarelli FR, Arcella A, Donofrio V, Diomedi Camassei F, Nozza P, Morra I, Massimino M, Pollo B, Giangaspero F, Antonelli M (2019) Role of immunohistochemistry in the identification of supratentorial C11ORF95-RELA fused ependymoma in routine neuropathology. Am J Surg Pathol 43:56\u201363. https:\/\/doi.org\/10.1097\/PAS.0000000000000979","journal-title":"Am J Surg Pathol"},{"key":"1238_CR27","doi-asserted-by":"publisher","first-page":"951","DOI":"10.1007\/s00401-020-02225-8","volume":"140","author":"JJ Zhu","year":"2020","unstructured":"Zhu JJ, Jillette N, Li X-N, Cheng AW, Lau CC (2020) C11orf95-RELA reprograms 3D epigenome in supratentorial ependymoma. Acta Neuropathol (Berl) 140:951\u2013960. https:\/\/doi.org\/10.1007\/s00401-020-02225-8","journal-title":"Acta Neuropathol (Berl)"},{"key":"1238_CR28","doi-asserted-by":"publisher","DOI":"10.1158\/2159-8290.CD-20-1052","author":"R Kupp","year":"2021","unstructured":"Kupp R, Ruff L, Terranova S, Nathan E, Ballereau S, Stark R, Sekhar Reddy Chilamakuri C, Hoffmann N, Wickham-Rahrmann K, Widdess M, Arabzade A, Zhao Y, Varadharajan S, Zheng T, Murugesan MK, Pfister SM, Kawauchi D, Pajtler KW, Deneen B, Mack SC, Masih KE, Gryder BE, Khan J, Gilbertson RJ (2021) ZFTA-translocations constitute ependymoma chromatin remodeling and transcription factors. Cancer Discov. https:\/\/doi.org\/10.1158\/2159-8290.CD-20-1052","journal-title":"Cancer Discov"}],"container-title":["Acta Neuropathologica Communications"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40478-021-01238-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s40478-021-01238-y\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40478-021-01238-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,8,13]],"date-time":"2021-08-13T04:09:52Z","timestamp":1628827792000},"score":1,"resource":{"primary":{"URL":"https:\/\/actaneurocomms.biomedcentral.com\/articles\/10.1186\/s40478-021-01238-y"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,8,13]]},"references-count":28,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["1238"],"URL":"https:\/\/doi.org\/10.1186\/s40478-021-01238-y","relation":{},"ISSN":["2051-5960"],"issn-type":[{"value":"2051-5960","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,8,13]]},"assertion":[{"value":"24 June 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"5 August 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 August 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare that they have no conflict of interest directly related to the topic of this article.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"135"}}