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The Challenge of “Monomorphic” Mucoepidermoid Carcinoma—Report of a Rare Case with Pure Spindle-Clear Cell Morphology

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Abstract

Background

Mucoepidermoid carcinoma is a malignant salivary gland tumor which, in most cases, is composed of variable proportions of mucous, epidermoid, and intermediate cells.

Methods

We report a case of parapharyngeal mucoepidermoid carcinoma with highly unusual (“monomorphic”) light microscopic features as well as atypical immunohistochemical properties. Molecular analysis was performed using the TruSight RNA fusion panel.

Results

The tumor featured heretofore undescribed histopathological features: sheets and nests composed of monomorphic neoplastic (plump spindle to epithelioid) cells with no mucous, intermediate, glandular/columnar, or any other cell type identified. The neoplastic cells displayed variable clear cell change and only expressed cytokeratin 7. Despite this non-classical morphology, the presence of the classical CRTC1::MAML2 fusion was demonstrated.

Conclusions

Mucoepidermoid carcinoma featuring a uniform (“monomorphic”) population of neoplastic cells is a novel observation. A confident diagnosis of mucoepidermoid carcinoma can be made upon detection of the CRTC1/3::MAML2 fusion. Our case increases the spectrum of histopathological appearances that mucoepidermoid carcinoma may display.

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Funding

This study was not supported by any funding. The authors did not receive support from any organization for the submitted work. The authors have no relevant financial or non-financial interests to disclose.

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Authors and Affiliations

  1. Department of Pathology, National University Health System, 5 Lower Kent Ridge Road, 119074, Singapore, Singapore

    Xinyi Qu, Bingcheng Wu & Fredrik Petersson

  2. Department of Pathology, Singapore General Hospital, Singapore, Singapore

    Edwin Jun Chen Chew & Sathiyamoorthy Selvarajan

  3. Department of Pathology, University of Erlangen, Erlangen, Germany

    Abbas Agaimy

Authors
  1. Xinyi Qu
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  2. Edwin Jun Chen Chew
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  3. Sathiyamoorthy Selvarajan
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  4. Bingcheng Wu
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  5. Abbas Agaimy
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  6. Fredrik Petersson
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Correspondence to Fredrik Petersson.

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Qu, X., Chew, E.J.C., Selvarajan, S. et al. The Challenge of “Monomorphic” Mucoepidermoid Carcinoma—Report of a Rare Case with Pure Spindle-Clear Cell Morphology. Head and Neck Pathol 17, 864–870 (2023). https://doi.org/10.1007/s12105-023-01547-9

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