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We investigated the prevalence and prognostic value of surface expression of PD\u2010L1, PD1, and CD30, copy number of 9p24.1 (PD\u2010L1 region), and mutations in <jats:italic>MYD88<\/jats:italic>, <jats:italic>CD79B<\/jats:italic>, <jats:italic>CARD11<\/jats:italic>, and <jats:italic>BTK<\/jats:italic> in a cohort of 116 patients, localized in the mediastinum (PMBL, <jats:italic>n<\/jats:italic>\u00a0=\u00a012), ear, nose and throat (ENT, <jats:italic>n<\/jats:italic>\u00a0=\u00a028), central nervous system (<jats:italic>n<\/jats:italic>\u00a0=\u00a029), testis (<jats:italic>n<\/jats:italic>\u00a0=\u00a07), breast (<jats:italic>n<\/jats:italic>\u00a0=\u00a04), stomach (<jats:italic>n<\/jats:italic>\u00a0=\u00a010), bone (<jats:italic>n<\/jats:italic>\u00a0=\u00a08), spleen (<jats:italic>n<\/jats:italic>\u00a0=\u00a02), and skin (<jats:italic>n<\/jats:italic>\u00a0=\u00a016). PD\u2010L1 expression is most frequent in PMBL (92%), followed by lymphomas originating in the stomach (57%), ENT (23%), and skin (18%). PD1 was expressed at low levels in less than 13% of PE\u2010DLBCL, while CD30 expression was found in 58% of PMBL. Mutation analysis revealed an unexpectedly high frequency of <jats:italic>MYD88<\/jats:italic> and <jats:italic>CD79B<\/jats:italic> mutations in ENT lymphomas (46% and 50%, respectively). <jats:italic>CARD11<\/jats:italic> mutations are rare but more frequently found in gastric lymphomas (30%), suggesting BTK resistance. Thirty\u2010four of 113 (30%) of the lymphomas harbored both <jats:italic>MYD88<\/jats:italic> and <jats:italic>CD79B<\/jats:italic> mutations. Lower overall and progression\u2010free survival rates were found for cases with <jats:italic>MYD88<\/jats:italic>, <jats:italic>CD79B<\/jats:italic>, and <jats:italic>BTK<\/jats:italic> mutations. These data confirm the biologic singularity of PE\u2010DLBCLs and provide some suggestions for targeted therapies.<\/jats:p>","DOI":"10.1002\/jha2.428","type":"journal-article","created":{"date-parts":[[2022,5,24]],"date-time":"2022-05-24T04:36:01Z","timestamp":1653366961000},"page":"688-697","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Targetable alterations in primary extranodal diffuse large B\u2010cell lymphoma"],"prefix":"10.1002","volume":"3","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5863-2305","authenticated-orcid":false,"given":"Stephanie E.","family":"Weissinger","sequence":"first","affiliation":[{"name":"Institute of Pathology University Hospital Ulm  Ulm Germany"},{"name":"Institute of Pathology Alb Fils Kliniken GmbH  G\u00f6ppingen 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