{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T18:52:20Z","timestamp":1789584740284,"version":"build-2803163510"},"reference-count":45,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2021,7,13]],"date-time":"2021-07-13T00:00:00Z","timestamp":1626134400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:p>Intracranial myxoid mesenchymal tumor (IMMT) is a recently described, extremely rare group of neoplasms characterized by fusions between the female\u2010expressed transcript (FET) family genes and the cAMP response element\u2010binding protein (CREB) family genes. Controversy persists regarding whether the tumor is a myxoid variant of angiomatoid fibrous histiocytoma or a completely distinct clinicopathological entity. Here, we report a case of IMMT arising in the posterior fossa in a 65\u2010year\u2010old woman with a history of breast cancer. We performed total removal of the tumor, which histologically demonstrated features characteristic of IMMT but also bore a partial resemblance to conventional angiomatoid fibrous histiocytoma. Immunohistochemically, tumor cells were diffusely positive for desmin, vimentin, cluster of differentiation (CD) 99 (CD99), glucose transporter\u20101, and cytokeratin (CK) 8\/18 (CK8\/18), and focally positive for CK7, epithelial membrane antigen, mucin 4, anaplastic lymphoma kinase, calponin, and CD68. Molecular genetic analysis revealed a fusion between the Ewing sarcoma breakpoint region 1 (EWSR1) gene (<jats:italic>EWSR1<\/jats:italic>) and the cAMP\u2010responsive element modulator (CREM) gene (<jats:italic>CREM<\/jats:italic>) called <jats:italic>EWSR1:CREM<\/jats:italic> fusion, which confirmed the diagnosis. The overlap of the pathological features of IMMTs and angiomatoid fibrous histiocytomas may support the recent theory that these tumors are two manifestations of a single entity. Moreover, our study indicated the broad spectrum of immunohistochemical phenotypes of these tumors, which should be noted during diagnosis. Further studies are needed to elucidate the histopathological concept, long\u2010term prognosis, optimal treatment strategy, and factors associated with the prognosis and therapeutic options of this condition.<\/jats:p>","DOI":"10.1111\/neup.12740","type":"journal-article","created":{"date-parts":[[2021,7,14]],"date-time":"2021-07-14T01:13:31Z","timestamp":1626225211000},"page":"315-323","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["A case of intracranial myxoid mesenchymal tumor with <i>EWSR1:CREM<\/i> fusion in an adult female: Extensive immunohistochemical evaluation"],"prefix":"10.1111","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7173-5915","authenticated-orcid":false,"given":"Atsushi","family":"Kambe","sequence":"first","affiliation":[{"name":"Division of Neurosurgery, Department of Brain and Neurosciences, Faculty of Medicine Tottori University  Tottori Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Kuwamoto","sequence":"additional","affiliation":[{"name":"Department of Pathology, Faculty of Medicine Tottori University  Tottori Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsuyoshi","family":"Shimizu","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of Brain and Neurosciences, Faculty of Medicine Tottori University  Tottori Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hidefumi","family":"Amisaki","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of Brain and Neurosciences, Faculty of Medicine Tottori University  Tottori Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Makoto","family":"Sakamoto","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of Brain and Neurosciences, Faculty of Medicine Tottori University  Tottori Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirotaka","family":"Inagaki","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Japanese Red Cross Tottori Hospital  Tottori Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masamichi","family":"Kurosaki","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of Brain and Neurosciences, Faculty of Medicine Tottori University  Tottori Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,7,13]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms19123784"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.5732\/cjc.013.10178"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10014-020-00359-x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-020-02885-7"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.20491"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-05-2811"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.20881"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e318227e4d2"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001331"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3181453451"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000788"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.13437"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1159\/000501695"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/nlz140"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.wneu.2020.07.057"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prp.2020.153239"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12504"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/nly039"},{"key":"e_1_2_7_20_1","first-page":"e12918","article-title":"Intracranial mesenchymal tumor with FET\u2010CREB fusion \u2013 A unifying diagnosis for the spectrum of intracranial myxoid mesenchymal tumors and angiomatoid fibrous histiocytoma\u2010like neoplasms","author":"Sloan EA","year":"2020","journal-title":"Brain Pathol"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.wneu.2016.09.059"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/nly050"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.3171\/2009.8.JNS081518"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1093\/neuros\/nyaa357"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000172"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1136\/jclinpath-2013-201857"},{"key":"e_1_2_7_27_1","first-page":"e4261","article-title":"Intracranial myxoid variant of angiomatoid fibrous histiocytoma: A case report and literature review","volume":"11","author":"Ghanbari N","year":"2019","journal-title":"Cureus"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10014-020-00389-5"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/nlaa114"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.25259\/SNI_282_2020"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00418-008-0435-6"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.wneu.2019.02.107"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001114"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001096"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13000-018-0752-6"},{"key":"e_1_2_7_36_1","article-title":"Clear cell tumor with melanocytic differentiation and MITF\u2010CREM translocation: A novel entity similar to clear cell sarcoma","author":"Fouchardiere A","year":"2021","journal-title":"Virchows Arch"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-020-0646-5"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-17-1856"},{"key":"e_1_2_7_39_1","article-title":"Gastric sclerosing epithelioid fibrosarcoma harboring a rare FUS\u2010CREM fusion","author":"Memon RA","year":"2020","journal-title":"Int J Surg Pathol"},{"key":"e_1_2_7_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.wneu.2020.07.015"},{"key":"e_1_2_7_41_1","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12900"},{"key":"e_1_2_7_42_1","doi-asserted-by":"publisher","DOI":"10.1111\/pin.13030"},{"key":"e_1_2_7_43_1","volume-title":"WHO Classification of Tumours of the Central Nervous System (WHO Health Organization Classification of Tumours) Revised 4th Ed","author":"Louis DN","year":"2016"},{"key":"e_1_2_7_44_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3182a0218f"},{"key":"e_1_2_7_45_1","doi-asserted-by":"publisher","DOI":"10.1002\/emmm.201100122"},{"key":"e_1_2_7_46_1","doi-asserted-by":"publisher","DOI":"10.1002\/emmm.201100121"}],"container-title":["Neuropathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/neup.12740","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/neup.12740","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/neup.12740","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,29]],"date-time":"2023-08-29T19:45:52Z","timestamp":1693338352000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/neup.12740"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,7,13]]},"references-count":45,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2021,8]]}},"alternative-id":["10.1111\/neup.12740"],"URL":"https:\/\/doi.org\/10.1111\/neup.12740","archive":["Portico"],"relation":{},"ISSN":["0919-6544","1440-1789"],"issn-type":[{"value":"0919-6544","type":"print"},{"value":"1440-1789","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,7,13]]},"assertion":[{"value":"2021-01-07","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-03-31","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-07-13","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}