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Review
. 2020 Feb 28;12(3):554.
doi: 10.3390/cancers12030554.

Management Recommendations for Merkel Cell Carcinoma-A Danish Perspective

Simon Naseri  1 , Torben Steiniche  1 , Morten Ladekarl  2 , Marie Louise Bønnelykke-Behrndtz  3 , Lisbet R Hölmich  4 , Seppo W Langer  5 , Alessandro Venzo  6 , Elizaveta Tabaksblat  7 , Siri Klausen  8 , Mathilde Skaarup Larsen  8 , Niels Junker  9 , Annette H Chakera  4
Affiliations
Review

Management Recommendations for Merkel Cell Carcinoma-A Danish Perspective

Simon Naseri et al. Cancers (Basel). .

Abstract

Merkel cell carcinoma (MCC) is a rare malignant neuroendocrine carcinoma of the skin with a poor prognosis and an apparent increase in incidence. Due to its rarity, evidence-based guidelines are limited, and there is a lack of awareness among clinicians. This review constitutes the consensus management recommendations developed by the Danish MCC expert group and is based on a systematic literature search. Patients with localized disease are recommended surgical excision and adjuvant radiotherapy to the primary site; however, this may be omitted in patients with MCC with low risk features. Patients with regional lymph node involvement are recommended complete lymph node removal and adjuvant radiotherapy in case of extracapsular disease. Metastatic disease was traditionally treated with chemotherapy, however, recent clinical trials with immune therapy have been promising. Immune checkpoint inhibitors targeting the programmed cell death protein 1(PD-1)/programmed death-ligand 1(PD-L1) axis should therefore be strongly considered as first-line treatment for fit patients. A 5-year follow-up period is recommended involving clinical exam every 3 months for 2 years and every 6 months for the following 3 years and PET-CT one to two times a year or if clinically indicated. These national recommendations are intended to offer uniform patient treatment and hopefully improve prognosis.

Keywords: Merkel cell carcinoma; diagnosis; guideline; review; treatment.

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Conflict of interest statement

Merck & Pfeizer sponsored the venue for the first and second Danish MCC Meeting and a 1-day MCC preceptorship at the Charité Hospital in Berlin, Germany. They had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.

Figures

Figure 1
Figure 1
A red, nodular primary MCC on the left hand.
Figure 2
Figure 2
Diagnostic workup for MCC patients.
See this image and copyright information in PMC

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