Sclerosing mucoepidermoid carcinoma of salivary glands

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 Herein, we report 25 unpublished cases of SMEC, two of them with prominent eosinophilia (2/25; 8%) and three with abundant IgG4-positive plasma cells (3/25; 12%).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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Publication Details

Journal
Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin
Published
2024-11-13
DOI
https://doi.org/10.1007/s00428-024-03970-x
Citations
5
Primary Topic
Salivary Gland Tumors Diagnosis and Treatment
Type
article
Field-Weighted Citation Impact
2.86

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Sclerosing mucoepidermoid carcinoma of salivary glands

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5 citations
Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin
Salivary Gland Tumors Diagnosis and Treatment
2.86
article

Sclerosing mucoepidermoid carcinoma of salivary glands

Stephan Ihrler, Ilmo Leivo, Ján Laco, Abbas Agaimy, Alena Skálová, Gisele de Rezende, Miguel Rito, Petr Šteiner, Martina Bradová, Senada Koljenović, Isabel Fonseca, Veronika Hájková, Bacem K. Othman, Roderick H. W. Simpson, Petr Grossmann, Michal Michal, Montse Gomà
article en
5 citations

Abstract

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.

Archiv für Pathologische Anatomie und Physiologie und für Klinische MedicinVol. 487(1)
University of Lisbon (PT), University of Antwerp (BE), University of Turku (FI), University of Calgary (CA), Friedrich-Alexander-Universität Erlangen-Nürnberg (DE), Charles University (CZ), Erasmus MC (NL), Turku University Hospital (FI), Bellvitge University Hospital (ES), Universitätsklinikum Erlangen (DE), Azienda Socio Sanitaria Territoriale Grande Ospedale Metropolitano Niguarda (IT), Antwerp University Hospital (BE), Biopticka Laborator (Czechia) (CZ), University Hospital Hradec Králové (CZ), Comprehensive Cancer Center Erlangen (DE)
Univerzita Karlova v Praze, European Commission, Turun Yliopistollinen Keskussairaala
Life below water
Openalex Percentile: Top 9%
Salivary Gland Tumors Diagnosis and Treatment
2.86
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