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A systematic PubMed search for sequencing studies of MZL was executed. All somatic mutations of the organs mentioned above were combined, uniformly annotated, and a dataset containing 25 publications comprising 6016 variants from 1663 patients was created. In splenic MZL, <jats:italic>KLF2<\/jats:italic> (18%, 103\/567) and <jats:italic>NOTCH2<\/jats:italic> (16%, 118\/725) were the most frequently mutated genes. Pulmonary and nodal MZL displayed recurrent mutations in chromatin-modifier-encoding genes, especially <jats:italic>KMT2D<\/jats:italic> (25%, 13\/51, and 20%, 20\/98, respectively). In contrast, ocular adnexal, gastric, and dura mater MZL had mutations in genes encoding for NF-\u03baB pathway compounds, in particular <jats:italic>TNFAIP3,<\/jats:italic> with 39% (113\/293), 15% (8\/55), and 45% (5\/11), respectively. Cutaneous MZL frequently had <jats:italic>FAS<\/jats:italic> mutations (63%, 24\/38), while MZL of the thyroid had a higher prevalence for <jats:italic>TET2<\/jats:italic> variants (61%, 11\/18). Finally, <jats:italic>TBL1XR1<\/jats:italic> (24%, 14\/58) was the most commonly mutated gene in MZL of the salivary glands. Mutations of distinct genes show origin-preferential distribution among nodal and splenic MZL as well as extranodal MZL at\/from different anatomic locations. Recognition of such mutational distribution patterns may help assigning MZL origin in difficult cases and possibly pave the way for novel more tailored treatment concepts.<\/jats:p>","DOI":"10.1007\/s00428-021-03186-3","type":"journal-article","created":{"date-parts":[[2021,9,8]],"date-time":"2021-09-08T03:30:28Z","timestamp":1631071828000},"page":"403-413","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":69,"title":["Mutational landscape of marginal zone B-cell lymphomas of various origin: organotypic alterations and diagnostic potential for assignment of organ origin"],"prefix":"10.1007","volume":"480","author":[{"given":"Visar","family":"Vela","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darius","family":"Juskevicius","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Dirnhofer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Menter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1100-3819","authenticated-orcid":false,"given":"Alexandar","family":"Tzankov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,9,8]]},"reference":[{"key":"3186_CR1","doi-asserted-by":"publisher","first-page":"275","DOI":"10.1177\/2040620712453595","volume":"3","author":"M Joshi","year":"2012","unstructured":"Joshi M, Sheikh H, Abbi K et al (2012) Marginal zone lymphoma: old, new, targeted, and epigenetic therapies. 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