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Herein, we report 25 unpublished cases of SMEC, two of them with prominent eosinophilia (2\/25; 8%) and three with abundant IgG4-positive plasma cells (3\/25; 12%). In our series of salivary SMEC, molecular analysis using fluorescence in situ hybridization (FISH) and\/or next-generation sequencing (NGS) provided evidence of <jats:italic>MAML2<\/jats:italic> gene rearrangement in 18 cases of the 21 analyzable cases tested (86%), while this gene locus was intact in 3 cases (14%). This study focuses on the diagnostic criteria of salivary SMEC given its challenge of abundant collagenous stroma, minimal residual neoplastic areas, and inconspicuous mucous cells. Follow-up data of our cases indicate that salivary SMECs have favorable outcomes. Molecular analysis for <jats:italic>MAML2<\/jats:italic> gene rearrangement suggests that SMECs of salivary glands represent a rare variant of conventional low-grade MECs of salivary glands. In contrast, SMECs of the thyroid gland are genetically distinct from salivary-type thyroid MECs.<\/jats:p>","DOI":"10.1007\/s00428-024-03970-x","type":"journal-article","created":{"date-parts":[[2024,11,13]],"date-time":"2024-11-13T22:09:45Z","timestamp":1731535785000},"page":"33-46","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Sclerosing mucoepidermoid carcinoma of salivary glands"],"prefix":"10.1007","volume":"487","author":[{"given":"Bacem Khalele","family":"Othman","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martina","family":"Bradov\u00e1","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roderick H. 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