Distant Pulmonary Metastasis in Microsecretory Adenocarcinoma of the Salivary Gland: A Case Report Highlighting Histological Variation and Aggressive Potential

Microsecretory adenocarcinoma (MSA) is a rare, recently defined, low-grade salivary gland neoplasm characterized by a recurrent <i>MEF2C::SS18</i> gene fusion and a generally indolent clinical course. Most tumors arise in the oral cavity, particularly the hard palate and buccal mucosa, and display microcystic and tubular architecture with bland cytological features and a consistent immunoprofile, including SOX10, S100, and p63 positivity, while p40 is negative. To date, fewer than 50 patients have been reported, with only 2 demonstrating high-grade histological features or distant metastasis. Herein, we report a 34-year-old woman with a tumor of the retromolar trigone, initially classified as polymorphous adenocarcinoma. Fifteen years later, the patient developed pulmonary metastases with distinct histomorphological features, including solid-cribriform differentiation and comedo-type necrosis. Molecular analysis identified an <i>MEF2C::SS18</i> gene fusion and an immunohistochemical profile consistent with MSA. This report highlights the histological variation and the potential for late metastatic progression in MSA, emphasizing the need for accurate molecular diagnosis and long-term clinical surveillance, even in tumors that appear histologically low-grade at initial presentation.

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Publication Details

Journal
International Journal of Surgical Pathology
Published
2025-11-03
DOI
https://doi.org/10.1177/10668969251384841
Primary Topic
Salivary Gland Tumors Diagnosis and Treatment
Type
article
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article

Distant Pulmonary Metastasis in Microsecretory Adenocarcinoma of the Salivary Gland: A Case Report Highlighting Histological Variation and Aggressive Potential

Sandra Hürlimann, Niels J. Rupp, Tamara Rordorf, Chrysa Stamou et al.
International Journal of Surgical Pathology
Salivary Gland Tumors Diagnosis and Treatment
article

Distant Pulmonary Metastasis in Microsecretory Adenocarcinoma of the Salivary Gland: A Case Report Highlighting Histological Variation and Aggressive Potential

Sandra Hürlimann, Niels J. Rupp, Tamara Rordorf, Chrysa Stamou, Sandra N. Freiberger, Mena Mansour, Irene Y. Chen, Aneeza Irfan, Hamza Irfan
article en

Abstract

Microsecretory adenocarcinoma (MSA) is a rare, recently defined, low-grade salivary gland neoplasm characterized by a recurrent <i>MEF2C::SS18</i> gene fusion and a generally indolent clinical course. Most tumors arise in the oral cavity, particularly the hard palate and buccal mucosa, and display microcystic and tubular architecture with bland cytological features and a consistent immunoprofile, including SOX10, S100, and p63 positivity, while p40 is negative. To date, fewer than 50 patients have been reported, with only 2 demonstrating high-grade histological features or distant metastasis. Herein, we report a 34-year-old woman with a tumor of the retromolar trigone, initially classified as polymorphous adenocarcinoma. Fifteen years later, the patient developed pulmonary metastases with distinct histomorphological features, including solid-cribriform differentiation and comedo-type necrosis. Molecular analysis identified an <i>MEF2C::SS18</i> gene fusion and an immunohistochemical profile consistent with MSA. This report highlights the histological variation and the potential for late metastatic progression in MSA, emphasizing the need for accurate molecular diagnosis and long-term clinical surveillance, even in tumors that appear histologically low-grade at initial presentation.

International Journal of Surgical PathologyVol. 34(2)
The University of Texas MD Anderson Cancer Center (US), University of Zurich (CH), Rashid Latif Medical College (PK), University of Rochester Medical Center (US), University Hospital of Zurich (CH), Luzerner Kantonsspital (CH), Hygeia Hospital (GR), Mitera Hospital (GR)
Openalex Percentile: Top 50%
Salivary Gland Tumors Diagnosis and Treatment
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