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Secretory Myoepithelial Carcinoma: A Histologic and Molecular Survey and a Proposed Nomenclature for Mucin Producing Signet Ring Tumors

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Abstract

Signet ring cell (mucin producing) adenocarcinoma is a rare low grade salivary gland malignancy. While currently designated as an adenocarcinoma, myoepithelial differentiation has been implied in previously reported cases. We herein perform a survey of our cases of signet ring cell adenocarcinoma and review the literature in order to refine categorization of this rare tumor. Five cases were retrieved. One was reclassified as a mammary analogue secretory carcinoma, leaving four that fulfilled the criteria for signet ring cell adenocarcinoma: the presence of prominent signet ring or vacuolated cells arranged in islands, interconnecting strands, cords or sheets in a myxoid or hyaline stroma, or pools of mucin. An extensive panel of histochemical and immunohistochemical stains and fluorescence in situ hybridization (FISH) (modeled after common phenotypes and molecular alterations seen in signet ring and myoepithelial tumors at other sites) was performed. The male-to-female ratio was 3:1. The mean age was 56 years (range 18–81). Sites involved included buccal mucosa (2), soft palate (1) and deep parotid (1). Perineural and angiolymphatic invasion were present in three and two cases respectively. One patient was lost to follow up and the remainder were alive and without disease at time of last follow up (mean 38 months). All cases showed mucicarmine positive vacuolated/signet ring cells embedded in a myxoid stroma. Three cases showed at least focal p63 staining and two cases showed positivity for calponin. Membranous E-cadherin was retained in all cases. FISH was negative for ETV6, EWSR1, and ALK1 rearrangements in all four cases. Based on the current series and the previously reported cases, it is evident that signet ring adenocarcinomas have a dual secretory and myoepithelial phenotype and thus as a whole more appropriately designated as ‘secretory myoepithelial carcinoma.’ They behave in a fairly indolent fashion and do not share the major molecular alterations seen in other signet ring and myoepithelial tumor types.

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Acknowledgements

We would like to thank the Electron Microscopy, Immunohistochemistry and In-situ Hybridization Laboratories for their excellent technical support. We would also like to thank Dr. Kathleen Vergona, at the University of Pittsburgh School of Dental Medicine Department of Oral Biology, for her assistance with the ultrastructural assessment of our cases.

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Authors and Affiliations

  1. Department of Histopathology, Sabah Hospital, Jamal Abdulnasir St., 3rd floor, Kuwait, Kuwait

    Jassem M. Bastaki

  2. Kuwait Cancer Control Center, Ministry of Health, Kuwait, Kuwait

    Jassem M. Bastaki

  3. Department of Pathology and Laboratory Medicine, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada

    Bibianna M. Purgina

  4. Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

    Sanja Dacic & Raja R. Seethala

  5. Department of Otolaryngology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

    Raja R. Seethala

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  1. Jassem M. Bastaki
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  2. Bibianna M. Purgina
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  3. Sanja Dacic
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  4. Raja R. Seethala
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Correspondence to Jassem M. Bastaki.

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Bastaki, J.M., Purgina, B.M., Dacic, S. et al. Secretory Myoepithelial Carcinoma: A Histologic and Molecular Survey and a Proposed Nomenclature for Mucin Producing Signet Ring Tumors. Head and Neck Pathol 8, 250–260 (2014). https://doi.org/10.1007/s12105-014-0518-8

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  • DOI: https://doi.org/10.1007/s12105-014-0518-8

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