Merkel cell carcinoma: An update and review : Current and future therapy - 28/02/18

Doi : 10.1016/j.jaad.2017.12.004

Tiffany L. Tello, MD ^a,^⁎ , Kathleen Coggshall, MD ^a, Sue S. Yom, MD, PhD, MAS ^b, Siegrid S. Yu, MD ^a
^a Department of Dermatology, University of California San Francisco, San Francisco, California
^b Department of Radiation Oncology, University of California San Francisco, San Francisco, California

^∗Reprint requests: Tiffany L. Tello, MD, University of California San Francisco, Department of Dermatology, 1701 Divisadero St, 3rd fl, San Francisco, CA 94115.University of California San FranciscoDepartment of Dermatology1701 Divisadero St3rd flSan FranciscoCA94115

Abstract

Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin associated with a high risk of local recurrence and distant metastases. It most commonly occurs on sun-exposed areas of white patients >65 years of age. The Merkel cell polyomavirus (MCV) is thought to be responsible for malignant transformation in approximately 80% of cases in the northern hemisphere, while ultraviolet radiation–induced DNA damage is implicated in MCV-negative tumors. The overall incidence of MCC is low, with approximately 1600 cases diagnosed annually in the United States. The rate is much higher in patients with lymphoproliferative malignancies, solid organ transplants, and HIV infection. The low overall incidence of this tumor makes it challenging to conduct prospective clinical trials with sufficient power. As a result, most management recommendations are based on case series, retrospective reviews, and expert opinion. The pathogenesis, diagnosis, and staging of MCC was discussed in the first article in this continuing medical education series. This article focuses on current management guidelines and promising new therapies in development. Because of the complexity, aggressive nature, and individuality of each case, MCC is best treated by a multidisciplinary team.

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Key words : immunotherapy, Merkel cell carcinoma, Merkel cell polyomavirus, neuroendocrine tumor

Abbreviations used : CLND, MCC, MCV, MHC, MMS, NCCN, PD-1, PD-L1, SLNB, TAg

Plan

Surgery

Management of the draining nodal basin

Sentinel lymph node biopsy

Clinical lymphadenopathy

Radiation therapy

Radiation monotherapy

Localized MCC

MCC with nodal disease

Immune checkpoint inhibitors

Targeted molecular therapy

Surveillance

	Funding sources: None.
	Dr Yom has received research grant funding from Genentech, Bristol-Myers Squibb, and Merck, was a consultant for BioMimetix, and has received royalties from UpToDate and Springer. Drs Tello, Coggshall, and Yu disclosed no conflicts of interest.
	Date of release: March 2018
	Expiration date: March 2021