TY - JOUR
T1 - Sclerosing mucoepidermoid carcinoma of salivary glands
AU - Othman, Bacem Khalele
AU - Bradova, Martina
AU - Simpson, Roderick H. W.
AU - Laco, Jan
AU - Agaimy, Abbas
AU - Rito, Miguel
AU - Ihrler, Stephan
AU - Steiner, Petr
AU - Grossmann, Petr
AU - Hajkova, Veronika
AU - de Rezende, Gisele
AU - Goma, Montse
AU - Koljenovic, Senada
AU - Fonseca, Isabel
AU - Michal, Michal
AU - Leivo, Ilmo
AU - Skalova, Alena
N1 - Publisher Copyright:
© The Author(s) 2024.
PY - 2024/11/14
Y1 - 2024/11/14
N2 - 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 Herein, we report 25 unpublished cases of SMEC, two of them with prominent eosinophilia (2/25; 8%) and three with abundant IgG4-positive plasma cells (3/25; 12%).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.
AB - 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 Herein, we report 25 unpublished cases of SMEC, two of them with prominent eosinophilia (2/25; 8%) and three with abundant IgG4-positive plasma cells (3/25; 12%).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.
UR - https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=eur_pure&SrcAuth=WosAPI&KeyUT=WOS:001353810300001&DestLinkType=FullRecord&DestApp=WOS_CPL
U2 - 10.1007/s00428-024-03970-x
DO - 10.1007/s00428-024-03970-x
M3 - Article
C2 - 39537991
SN - 0945-6317
JO - Virchows Archiv
JF - Virchows Archiv
ER -