Head and Neck Pathology

, Volume 12, Issue 1, pp 31–43 | Cite as

Update on Merkel Cell Carcinoma

Proceedings of the North American Society of Head and Neck Pathology Companion Meeting, March 18, 2018, Vancouver, British Columbia, Canada

Abstract

Merkel cell carcinoma (MCC) is an aggressive cutaneous neuroendocrine carcinoma. Incidence of MCC continues to rise, and risk factors include advanced age, pale skin, chronic sun exposure, and immune suppression. Diagnosing MCC utilizes a combination of morphology and immunohistochemistry. Merkel cell polyomavirus (MCPyV) is present in approximately 70–80% of MCCs and represents a key pathogenic driver in those MCCs. In contrast, MCPyV-negative MCCs arise through progressive accumulation of ultraviolet-light induced somatic mutations. Staging of MCC proceeds according to the American Joint Commission on Cancer (AJCC) 8th Edition, which utilizes features of the primary tumor together with regional lymph node(s) (clinically and/or pathologically detected) and/or distant metastases. Many potentially useful biomarkers have been studied to refine risk stratification in MCC. In recent years, the host immune infiltrate has been leveraged as immune checkpoint blockade has emerged as an efficacious mode of treatment for patients with advanced MCC.

Keywords

Merkel cell carcinoma Merkel cell polyoma virus Pathogenesis Prognostic factors Staging 

Notes

Compliance with Ethical Standards

Conflict of interest

MTT has received an honorarium from Myriad Genetics, Seattle Genetics and Novartis for Advisory Boards. MTT declares no relevant conflicts of interest to the material presented herein.

Ethical Approval

This article does not contain any studies with human participants performed by any of the authors. This article does not contain any studies with animals performed by any of the authors.

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of PathologyThe University of Texas MD Anderson Cancer CenterHoustonUSA
  2. 2.Department of Translational and Molecular PathologyThe University of Texas MD Anderson Cancer CenterHoustonUSA

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