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Herein, we describe five new IMT cases from four females and one male with three harboring an <jats:italic>EWSR1::CREM<\/jats:italic> fusion and two featuring an <jats:italic>EWSR1::ATF1<\/jats:italic> fusion. Uniform manifold approximation and projection of DNA methylation array data placed two cases to the methylation class \u201cIMT, subclass B\u201d, one to \u201cmeningioma-benign\u201d and one to \u201cmeningioma-intermediate\u201d. A literature review identified 74 cases of IMTs (current five cases included) with a median age of 23\u00a0years (range 4\u201379\u00a0years) and a slight female predominance (female\/male ratio\u2009=\u20091.55). Among the confirmed fusions, 25 (33.8%) featured an <jats:italic>EWSR1::ATF1<\/jats:italic> fusion, 24 (32.4%) <jats:italic>EWSR1::CREB1<\/jats:italic>, 23 (31.1%) <jats:italic>EWSR1::CREM<\/jats:italic>, one (1.4%) <jats:italic>FUS::CREM<\/jats:italic>, and one (1.4%) <jats:italic>EWSR1::CREB3L3<\/jats:italic>. Among 66 patients with follow-up information available (median: 17\u00a0months; range: 1\u2013158\u00a0months), 26 (39.4%) experienced progression\/recurrences (median 10.5\u00a0months; range 0\u2013120\u00a0months). Ultimately, three patients died of disease, all of whom underwent a subtotal resection for an <jats:italic>EWSR1::ATF1<\/jats:italic> fusion-positive tumor. Outcome analysis revealed subtotal resection as an independent factor associated with a significantly shorter progression free survival (PFS; median: 12\u00a0months) compared with gross total resection (median: 60\u00a0months; <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). A younger age (&lt;\u200914\u00a0years) was associated with a shorter PFS (median: 9\u00a0months) compared with an older age (median: 49\u00a0months; <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.05). Infratentorial location was associated with a shorter overall survival compared with supratentorial (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.05). In addition, the <jats:italic>EWSR1::ATF1<\/jats:italic> fusion appeared to be associated with a shorter overall survival compared with the other fusions (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.05). In conclusion, IMT is a locally aggressive tumor with a high recurrence rate. Potential risk factors include subtotal resection, younger age, infratentorial location, and possibly <jats:italic>EWSR1::ATF1<\/jats:italic> fusion. Larger case series are needed to better define prognostic determinants in these tumors.<\/jats:p>","DOI":"10.1186\/s40478-024-01721-2","type":"journal-article","created":{"date-parts":[[2024,1,30]],"date-time":"2024-01-30T11:02:15Z","timestamp":1706612535000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Potential prognostic determinants for FET::CREB fusion-positive intracranial mesenchymal tumor"],"prefix":"10.1186","volume":"12","author":[{"given":"Frank M.","family":"Mezzacappa","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frankie K.","family":"Smith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weiwei","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Gard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fatmagul Kusku","family":"Cabuk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ignancio","family":"Gonzalez-Gomez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hector L.","family":"Monforte","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiancong","family":"Liang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Omkar","family":"Singh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martha M.","family":"Quezado","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth D.","family":"Aldape","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Murat","family":"Gokden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia A.","family":"Bridge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6280-5320","authenticated-orcid":false,"given":"Jie","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,1,30]]},"reference":[{"key":"1721_CR1","doi-asserted-by":"publisher","first-page":"504","DOI":"10.1097\/pas.0000000000002022","volume":"47","author":"AM Acosta","year":"2023","unstructured":"Acosta AM, Bridge JA, Dal Cin PS, Sholl LM, Cornejo KM, Fletcher CDM, Ulbright TM (2023) Inflammatory and nested testicular sex cord tumor: a novel neoplasm with aggressive clinical behavior and frequent EWSR1::ATF1 gene fusions. 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