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Microsecretory Adenocarcinoma of Salivary Glands: An Expanded Series of 24 Cases

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Abstract

Microsecretory adenocarcinoma (MSA) is a recently described salivary gland tumor with a characteristic histologic and immunophenotypic profile and recurrent MEF2C-SS18 fusions. Because only six cases of MSA have been published, its complete clinicopathologic spectrum is unclear, and its biologic behavior has not been documented. Here, we present an updated and expanded experience of 24 MSA cases. All cases of MSA were obtained from the authors’ files. Immunohistochemistry for S100, SOX10, p63, p40, SMA, calponin, and mammaglobin was performed. Molecular analysis was performed by targeted RNA sequencing, SS18 break apart fluorescence in situ hybridization, and/or reverse transcriptase polymerase chain reaction for MEF2C-SS18 fusion. Clinical follow-up was obtained from medical records. A total of 24 MSA cases were collected, from 13 women and 11 men, ranging from 17 to 83 years (mean 49.5 years). The vast majority (23 of 24) arose in the oral cavity, with the palate (n = 14) and buccal mucosa (n = 6) as the most frequent subsites. Tumors showed consistent histologic features including: (1) microcystic tubules, (2) flattened intercalated duct-like cells, (3) monotonous oval hyperchromatic nuclei, (4) abundant basophilic luminal secretions, (5) fibromyxoid stroma, and (6) circumscribed borders with subtle infiltration. The tumors were very consistently positive for S100 (24 of 24), p63 (24 of 24), and SOX10 (14 of 14) and negative for p40 (0 of 21), calponin (0 of 12) and mammaglobin (0 of 16), while SMA (4 of 20) was variable. MEF2C-SS18 fusion was demonstrated in 21 of 24 cases; in the remaining 3 cases with insufficient RNA, SS18 break apart FISH was positive. Treatment information was available in 17 cases, all of which were managed with surgery only. In 14 cases with follow-up (1–216 months, mean 30), no cases recurred or metastasized. MSA is a distinct salivary gland neoplasm with remarkably consistent clinical, histologic, immunophenotypic, and genetic features that generally behaves in an indolent manner following surgery alone. These observations solidify MSA as a unique, low-grade salivary gland carcinoma that warrants inclusion in the next version of the WHO classification of head and neck tumors.

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Funding

This study was funded by the Jane B. and Edwin P. Jenevein M.D Endowment for Pathology at UT Southwestern Medical Center. No external funding was obtained for this study.

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Authors and Affiliations

  1. Department of Pathology, UT Southwestern Medical Center, Dallas, TX, USA

    Justin A. Bishop & Jeffrey Gagan

  2. Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, CA, USA

    Dipti P. Sajed & Jeffrey F. Krane

  3. Department of Pathology, University Health Network, Toronto, ON, Canada

    Ilan Weinreb

  4. Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada

    Ilan Weinreb

  5. Department of Pathology & Laboratory Medicine, Mount Sinai Hospital, Toronto, Canada

    Brendan C. Dickson

  6. Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada

    Brendan C. Dickson

  7. Department of Diagnostic Sciences, School of Dental Medicine, University of Pittsburgh, Pittsburgh, USA

    Elizabeth A. Bilodeau

  8. Institute of Pathology, University Hospital of Erlangen, Erlangen, Germany

    Abbas Agaimy

  9. Department of Translational Research and New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy

    Alessandro Franchi

  10. Unit of Oral and Maxillofacial Pathology, School of Clinical Dentistry, 19 Claremont Crescent, Sheffield, S10 2TA, UK

    Syed Ali Khurram

  11. Department of Cellular Pathology, University Hospitals of Leicester NHS Trust, Leicester, UK

    Philip Da Forno

  12. Department of Pathology and Laboratory Medicine, Long School of Medicine, UT Health, San Antonio, TX, USA

    Juliana Robledo

  13. Division of Oral and Maxillofacial Pathology, The Ohio State University College of Dentistry, Columbus, OH, USA

    John R. Kalmar

  14. Division of Oral and Maxillofacial Pathology, The University of Tennessee Health Science Center College of Dentistry, Memphis, TN, USA

    Sarah Aguirre

  15. Department of Oral Diagnostic Sciences, School of Dental Medicine, State University of New York at Buffalo, Buffalo, NY, USA

    Jose Luis Tapia

  16. Department of Pathology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark

    Katalin Kiss

  17. Department of Diagnostic Sciences, Louisiana State University Health Sciences Center School of Dentistry, New Orleans, LA, USA

    Kitrina Cordell & Molly Rosebush

  18. Department of Histopathology, Queen Victoria Hospital, Holtye Road, East Grinstead, West Sussex, RH19 3DZ, UK

    A. William Barrett

  19. Department Oral & Maxillofacial Surgery, School of Dentistry, University of Washington, Seattle, WA, USA

    Dolphine Oda

  20. Department of Pathology, Virginia Mason Hospital & Seattle Medical Center, Seattle, WA, USA

    Adel Assaad

  21. Department of Anatomic Pathology, Tokyo Medical University, Tokyo, Japan

    Toshitaka Nagao

  22. Department of Diagnostic Pathology, Kumamoto University Hospital, Kumamoto, Japan

    Fumi Kawakami

  23. Departments of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan

    Masato Nakaguro

  24. Department of Pathology Mount Sinai Brooklyn, Icahn School of Medicine at Mount Sinai Hospital, New York, NY, USA

    Ismail Zahir

  25. Department of Pathology and Laboratory Medicine, University of Louisville, Louisville, KY, USA

    Kristina Wakeman

  26. DERMPATH Muenchen, Munich, Germany

    Stephan Ihrler

  27. Pathology Department, L’Hôtel-Dieu de Québec, Centre Hospitalier Universitaire de Québec, Laval University, Quebec, Canada

    Jacinthe Chenevert

  28. Division of Diagnostic and Surgical Sciences, School of Dentistry, University of California, Los Angeles, CA, USA

    Yi-Ling Lin

  29. Department of Pathology, Icahn School of Medicine at Mount Sinai Hospital, New York, NY, USA

    William H. Westra

  30. Department of Pathology, The Johns Hopkins Hospital, Baltimore, MD, USA

    Lisa M. Rooper

Authors
  1. Justin A. Bishop
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  2. Dipti P. Sajed
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  3. Ilan Weinreb
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  4. Brendan C. Dickson
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  5. Elizabeth A. Bilodeau
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  6. Abbas Agaimy
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  7. Alessandro Franchi
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  8. Syed Ali Khurram
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  9. Philip Da Forno
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  10. Juliana Robledo
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  11. John R. Kalmar
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  12. Sarah Aguirre
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  13. Jeffrey F. Krane
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  14. Jose Luis Tapia
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  15. Katalin Kiss
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  16. Kitrina Cordell
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  17. Molly Rosebush
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  18. A. William Barrett
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  19. Dolphine Oda
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  20. Adel Assaad
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  21. Toshitaka Nagao
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  22. Fumi Kawakami
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  23. Masato Nakaguro
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  24. Ismail Zahir
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  25. Kristina Wakeman
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  26. Stephan Ihrler
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  27. Jacinthe Chenevert
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  28. Yi-Ling Lin
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  29. William H. Westra
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  30. Jeffrey Gagan
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  31. Lisa M. Rooper
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Corresponding author

Correspondence to Justin A. Bishop.

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All authors declare that he/she has no conflict of interest as it relates to this research project.

Ethical Approval

All procedures performed in this retrospective data analysis involving human participants were in accordance with the ethical standards of the institutional review board (IRB 112017–073), which did not require informed consent.

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Bishop, J.A., Sajed, D.P., Weinreb, I. et al. Microsecretory Adenocarcinoma of Salivary Glands: An Expanded Series of 24 Cases. Head and Neck Pathol 15, 1192–1201 (2021). https://doi.org/10.1007/s12105-021-01331-7

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  • DOI: https://doi.org/10.1007/s12105-021-01331-7

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