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Merkel Cell Carcinoma: Updates on Pathogenesis, Diagnosis, and Management

  • Skin Cancer (A Marghoob and M Marchetti, Section Editors)
  • Published:
Current Dermatology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

To describe updates on the pathogenesis, diagnosis, and management of Merkel cell carcinoma (MCC).

Recent Findings

Sequencing studies revealed that MCCs have either a low mutational burden and integrated Merkel cell polyomavirus (MCPyV), or they have a high number of ultraviolet-associated somatic mutations and no MCPyV. Clinically, prognosis was better for stage III MCC of unknown primary than known primary. Similarly, lack of immunosuppression conferred better prognosis. The immunogenicity of MCC was reflected in high response rates to PD-1/PD-L1 checkpoint inhibitors.

Summary

MCC is a rare but aggressive neuroendocrine skin cancer associated with advanced age and immunosuppression. Approximately 80% of MCCs are MCPyV driven, whereas MCPyV-negative tumors have mutations in genes such as p53 and RB1. MCC is highly immunogenic, and recently, the anti-PD-L1 antibody avelumab was approved to treat metastatic MCC. Here, we summarized features of the pathogenesis, diagnosis, and management of MCC.

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Funding

This research was supported by the NIH Intramural Research Program, Center of Cancer Research, National Cancer Institute.

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  1. Dermatology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Building 10, Room 12N240C, Bethesda, MD, 20892-1908, USA

    Jannett Nguyen, Natasha Hill & Isaac Brownell

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  1. Jannett Nguyen
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  2. Natasha Hill
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Correspondence to Isaac Brownell.

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Dr. Brownell reports work prepared as part of official duties as a US government employee for Intramural Research Program, CCR, NCI, during the conduct of the study.

Jannett Nguyen and Natasha Hill declare that they have no conflict of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on Skin Cancer

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Nguyen, J., Hill, N. & Brownell, I. Merkel Cell Carcinoma: Updates on Pathogenesis, Diagnosis, and Management. Curr Derm Rep 7, 158–168 (2018). https://doi.org/10.1007/s13671-018-0221-1

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