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Merkel Cell Carcinoma of the Buccal Mucosa and Lower Lip

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Abstract

Merkel cell carcinoma (MCC) is an uncommon relatively aggressive neuroendocrine dermal neoplasm first described in 1972 as a tumor of the sun exposed skin. Although most MCC affect the skin of the head and neck, rare primarily oral mucosal cases have been documented. Merkel cells are nondendritic neuroendocrine cells that are found not only in the skin but also the oral mucosa and give rise to MCC. Neuroendocrine cells may be found as aggregates in organs or as diffuse or isolated cells within organs and their epithelial lining. They contain peptide hormones and biogenic amines and occur in two forms: dendritic, which are not associated with nerve fibers and non-dendritic, which are associated with nerve fibers. Merkel cells as well as MCC express simple epithelium-type Cytokeratins (8, 18, 19, 20), neurosecretory substances; chromogranin A, synaptophysin, neuron-specific enolase (NSE), adhesion molecules, and villin (intermediate filament). Though weakly, they also express neural markers such as S-100 protein. Cytokeratin 20, and Cluster of differentiation 56, are the two key diagnostic markers for Merkel cells and MCC. Etiology includes UV radiation, the recently described Merkel cell polyomavirus, and long term systemic immunosuppression. The cutaneous and mucosal variants of MCC are considered aggressive tumors with a high risk for local recurrence and metastasis and should be considered in the differential diagnosis of head and neck mucosal lesions. We present two cases of primary Merkel cell carcinoma, one on the buccal mucosa and the other on the lower lip, and discuss the salient histologic, immunohistochemical and clinical features.

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Authors and Affiliations

  1. Department of Oral and Maxillofacial Surgery and Diagnostic Sciences, University of Florida College of Dentistry, Gainesville, FL, 32610, USA

    Mohammed N. Islam, Molly Housley Smith & Indraneel Bhattacharyya

  2. Department of Oral Diagnostic Sciences, Creighton University, School of Dentistry, Omaha, NE, USA

    Hardeep Chehal

  3. Indiana University School of Medicine, Evansville, IN, USA

    Sarah Islam

Authors
  1. Mohammed N. Islam
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  2. Hardeep Chehal
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  3. Molly Housley Smith
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  4. Sarah Islam
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Correspondence to Mohammed N. Islam.

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Mohammed N. Islam, Hardeep Chehal, Molly Housley Smith, Sarah Islam and Indraneel Bhattacharyya declares that they have no conflict of interest.

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Islam, M.N., Chehal, H., Smith, M.H. et al. Merkel Cell Carcinoma of the Buccal Mucosa and Lower Lip. Head and Neck Pathol 12, 279–285 (2018). https://doi.org/10.1007/s12105-017-0859-1

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  • DOI: https://doi.org/10.1007/s12105-017-0859-1

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