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Nat Rev Clin Oncol. 2018 Dec;15(12):763-776. doi: 10.1038/s41571-018-0103-2.

The biology and treatment of Merkel cell carcinoma: current understanding and research priorities.

Author information

1
Department of Pathology, University of Michigan Medical School, Ann Arbor, MI, USA.
2
Department of Dermatology, University of Michigan Medical School, Ann Arbor, MI, USA.
3
Cancer Virology Program, University of Pittsburgh, Pittsburgh, PA, USA.
4
Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA, USA.
5
Department of Medicine, Division of Dermatology, University of Washington, Seattle, WA, USA.
6
Department of Melanoma Medical Oncology, Division of Cancer Medicine, MD Anderson Cancer Center, Houston, TX, USA.
7
Dermatology Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) and National Cancer Institute (NCI), NIH, Bethesda, MD, USA. isaac.brownell@nih.gov.

Abstract

Merkel cell carcinoma (MCC) is a rare and aggressive skin cancer associated with advanced age and immunosuppression. Over the past decade, an association has been discovered between MCC and either integration of the Merkel cell polyomavirus, which likely drives tumorigenesis, or somatic mutations owing to ultraviolet-induced DNA damage. Both virus-positive and virus-negative MCCs are immunogenic, and inhibition of the programmed cell death protein 1 (PD-1)-programmed cell death 1 ligand 1 (PD-L1) immune checkpoint has proved to be highly effective in treating patients with metastatic MCC; however, not all patients have a durable response to immunotherapy. Despite these rapid advances in the understanding and management of patients with MCC, many basic, translational and clinical research questions remain unanswered. In March 2018, an International Workshop on Merkel Cell Carcinoma Research was held at the US National Cancer Institute, at which academic, government and industry experts met to identify the highest-priority research questions. Here, we review the biology and treatment of MCC and report the consensus-based recommendations agreed upon during the workshop.

PMID:
30287935
PMCID:
PMC6319370
DOI:
10.1038/s41571-018-0103-2
[Indexed for MEDLINE]
Free PMC Article

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