{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T21:35:38Z","timestamp":1783114538662,"version":"3.54.6"},"reference-count":67,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2022,12,25]],"date-time":"2022-12-25T00:00:00Z","timestamp":1671926400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Genes Chromosomes &amp; Cancer"],"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A focal adenomatoid\u2010microcystic pattern is not uncommon in peritoneal mesothelioma, but tumors composed almost exclusively of this pattern are distinctly rare and have not been well characterized. A small subset of mesotheliomas (mostly in children and young adults) are characterized by gene fusions including <jats:italic>EWSR1\/FUS::ATF1<\/jats:italic>, <jats:italic>EWSR1::YY1<\/jats:italic>, and <jats:italic>NTRK<\/jats:italic> and <jats:italic>ALK<\/jats:italic> rearrangements, and often have epithelioid morphology. Herein, we describe five peritoneal mesothelial neoplasms (identified via molecular screening of seven histologically similar tumors) that are pure adenomatoid\/microcystic in morphology and unified by the presence of an <jats:italic>NR4A3<\/jats:italic> fusion. Patients were three males and two females aged 31\u201370\u2009years (median, 40\u2009years). Three presented with multifocal\/diffuse and two with a localized disease. The size of the individual lesions ranged from 1.5 to 8\u00a0cm (median, 4.7). The unifocal lesions originated in the small bowel mesentery and the mesosigmoid. Treatment included surgery, either alone (three) or combined with hyperthermic intraperitoneal chemotherapy (two), and neoadjuvant or adjuvant chemotherapy (one case each). At the last follow\u2010up (6\u201313\u2009months), all five patients were alive and disease\u2010free. All tumors were morphologically similar, characterized by extensive sieve\u2010like microcystic growth with bland\u2010looking flattened cells lining variably sized microcystic spaces and lacked a conventional epithelioid or sarcomatoid component. Immunohistochemistry confirmed mesothelial differentiation, but most cases showed limited expression of D2\u201040 and calretinin. Targeted RNA sequencing revealed an <jats:italic>NR4A3<\/jats:italic> fusion (fusion partners were <jats:italic>EWSR1<\/jats:italic> in three cases and <jats:italic>CITED2<\/jats:italic> and <jats:italic>NIPBL<\/jats:italic> in one case each). The nosology and behavior of this morphomolecularly defined novel peritoneal mesothelial neoplasm of uncertain biological potential and its distinction from adenomatoid variants of conventional mesothelioma merit further delineation as more cases become recognized.<\/jats:p>","DOI":"10.1002\/gcc.23118","type":"journal-article","created":{"date-parts":[[2022,12,16]],"date-time":"2022-12-16T12:03:05Z","timestamp":1671192185000},"page":"256-266","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["<scp>NR4A3<\/scp> fusions characterize a distinctive peritoneal mesothelial neoplasm of uncertain biological potential with pure adenomatoid\/microcystic morphology"],"prefix":"10.1002","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0445-8161","authenticated-orcid":false,"given":"Abbas","family":"Agaimy","sequence":"first","affiliation":[{"name":"Institute of Pathology, Friedrich Alexander University Erlangen\u2010N\u00fcrnberg, University Hospital  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9098-8416","authenticated-orcid":false,"given":"Luka","family":"Brcic","sequence":"additional","affiliation":[{"name":"Diagnostic and Research Institute of Pathology, Medical University of Graz  Graz Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurence M.","family":"Briski","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine University of Miami Miller School of Medicine  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8568-1591","authenticated-orcid":false,"given":"Yin P.","family":"Hung","sequence":"additional","affiliation":[{"name":"Department of Pathology Massachusetts General Hospital  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Michal","sequence":"additional","affiliation":[{"name":"Department of Pathology Charles University, Faculty of Medicine in Plzen  Pilsen Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michal","family":"Michal","sequence":"additional","affiliation":[{"name":"Department of Pathology Charles University, Faculty of Medicine in Plzen  Pilsen Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. Petur","family":"Nielsen","sequence":"additional","affiliation":[{"name":"Department of Pathology Massachusetts General Hospital  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Stoehr","sequence":"additional","affiliation":[{"name":"Institute of Pathology, Friedrich Alexander University Erlangen\u2010N\u00fcrnberg, University Hospital  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew E.","family":"Rosenberg","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine University of Miami Miller School of Medicine  Miami Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,12,25]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-021-00895-7"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.5858\/arpa.2017-0124-RA"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1309\/2H0NVRERPP2LJDUA"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001418"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001924"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0010612"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/JTO.0000000000000493"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-16-1971"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.3520"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1158\/2159-8290.CD-18-0804"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12967-015-0485-1"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtho.2016.12.019"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2016.188"},{"key":"e_1_2_7_15_1","first-page":"1993","volume-title":"BAP1 Tumor Predisposition Syndrome. 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