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These lymphomas are classified according to gene expression profiling (GEP) into germinal center B-cell (GCB) and activated B-cell type (ABC). Recent studies have suggested new subtypes of large B-cell lymphoma, based on genetic and molecular alterations, among them is large B-cell lymphoma with <jats:italic>IRF4<\/jats:italic>-rearrangement (LBCL-<jats:italic>IRF4<\/jats:italic>). We used fluorescence in situ hybridization (FISH), GEP (using the DLBCL COO assay by HTG Molecular Inc), and next generation sequencing (NGS) to comprehensively characterize 30 cases of LBCLs located in Waldeyer\u2019s ring in adult patients and to identify LBCL<jats:italic>-IRF4<\/jats:italic>. FISH revealed breaks of <jats:italic>IRF4<\/jats:italic> in 2\/30 cases (6.7%), <jats:italic>BCL2<\/jats:italic> breaks in 6\/30 cases (20.0%), and IGH breaks in 13\/29 cases (44.8%). GEP classified 14 cases each as GCB or ABC subtype, and 2 cases remained unclassified; this was concordant with the immunohistochemistry (IHC) in 25\/30 cases (83.3%). A subgrouping, based on GEP, was performed: group 1 included 14 GCB cases with the most frequent mutations in <jats:italic>BCL2<\/jats:italic> and <jats:italic>EZH2<\/jats:italic> in 6\/14 cases (42.8%). The two cases with <jats:italic>IRF4<\/jats:italic> rearrangement were assigned to this group by GEP and showed <jats:italic>IRF4<\/jats:italic> mutations, supporting the diagnosis of LBCL-<jats:italic>IRF4<\/jats:italic>. Group 2 included 14 ABC cases; the most frequent mutations were <jats:italic>CD79B<\/jats:italic> and <jats:italic>MYD88<\/jats:italic> identified in 5\/14 patients (35.7%). Group 3 included 2 unclassifiable cases in which no molecular patterns were detected. Overall, LBCLs of Waldeyer\u2019s ring in adult patients are a heterogeneous group, including LBCL-<jats:italic>IRF4<\/jats:italic>, which shares several features with cases in the pediatric population.<\/jats:p>","DOI":"10.1007\/s00428-023-03516-7","type":"journal-article","created":{"date-parts":[[2023,2,22]],"date-time":"2023-02-22T00:04:25Z","timestamp":1677024265000},"page":"551-560","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Prevalence of IRF4 rearrangement in large B-cell lymphomas of the Waldeyer\u2019s ring in adults"],"prefix":"10.1007","volume":"482","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7334-1565","authenticated-orcid":false,"given":"Sebastian","family":"Streich","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4236-5799","authenticated-orcid":false,"given":"Leonie","family":"Frauenfeld","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franziska","family":"Otto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Mankel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7732-0788","authenticated-orcid":false,"given":"Irina","family":"Bonzheim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5496-293X","authenticated-orcid":false,"given":"Falko","family":"Fend","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7156-5365","authenticated-orcid":false,"given":"Leticia","family":"Quintanilla-Martinez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,2,22]]},"reference":[{"key":"3516_CR1","doi-asserted-by":"publisher","first-page":"503","DOI":"10.1038\/35000501","volume":"403","author":"AA Alizadeh","year":"2000","unstructured":"Alizadeh AA, Eisen MB, Davis RE, Ma C, Lossos IS, Rosenwald A, Boldrick JC, Sabet H, Tran T, Yu X, Powell JI, Yang L, Marti GE, Moore T, Hudson J, Lu L, Lewis DB, Tibshirani R, Sherlock G, Chan WC, Greiner TC, Weisenburger DD, Armitage JO, Warnke R, Levy R, Wilson W, Grever MR, Byrd JC, Botstein D, Brown PO, Staudt LM (2000) Distinct types of diffuse large B-cell lymphoma identified by gene expression profiling. 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