Sclerosing mucoepidermoid carcinoma of salivary glands
: Othman, Bacem Khalele; Bradová, Martina; Simpson, Roderick H. W.; Laco, Jan; Agaimy, Abbas; Rito, Miguel; Ihrler, Stephan; Steiner, Petr; Grossmann, Petr; Hájková, Veronika; de Rezende, Gisele; Goma, Montse; Koljenovic, Senada; Fonseca, Isabel; Michal, Michal; Leivo, Ilmo; Skalova, Alena
Publisher: Springer Science and Business Media LLC
: 2024
: Virchows Archiv
: Virchows Archiv
: Virchows Arch
: 0945-6317
: 1432-2307
DOI: https://doi.org/10.1007/s00428-024-03970-x
: https://doi.org/10.1007/s00428-024-03970-x
: https://research.utu.fi/converis/portal/detail/Publication/470942866
Sclerosing mucoepidermoid carcinoma (SMEC) of the salivary glands is a rare variant of low-grade mucoepidermoid carcinoma with scanty cellular atypia characterized by marked fibrosis/sclerosis and a rich inflammatory infiltrate. Herein, we report 25 unpublished cases of SMEC, two of them with prominent eosinophilia (2/25; 8%) and three with abundant IgG4-positive plasma cells (3/25; 12%).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 MAML2 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 MAML2 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.
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Open access publishing supported by the National Technical Library in Prague. This study was supported by study grant SVV 260652 from the Ministry of Education of the Czech Republic (NK), the Cooperation Program—research area SURG from the Charles University, Czech Republic (MB, AS), the project National Institute for Cancer Research—NICR (Programme EXCELES, ID Project No. LX22NPO5102)—funded by the European Union—Next Generation EU (AS, MB), and Turku University Hospital Fund, Maritza and Reino Salonen Foundation, and the Finnish Cancer Society, Finland (IL).
