Management Recommendations for Merkel Cell Carcinoma—A Danish Perspective

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 managem...

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Main Authors: Simon Naseri, Torben Steiniche, Morten Ladekarl, Marie Louise Bønnelykke-Behrndtz, Lisbet R. Hölmich, Seppo W. Langer, Alessandro Venzo, Elizaveta Tabaksblat, Siri Klausen, Mathilde Skaarup Larsen, Niels Junker, Annette H. Chakera
Format: Article
Language:English
Published: MDPI AG 2020-02-01
Series:Cancers
Subjects:
Online Access:https://www.mdpi.com/2072-6694/12/3/554
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author Simon Naseri
Torben Steiniche
Morten Ladekarl
Marie Louise Bønnelykke-Behrndtz
Lisbet R. Hölmich
Seppo W. Langer
Alessandro Venzo
Elizaveta Tabaksblat
Siri Klausen
Mathilde Skaarup Larsen
Niels Junker
Annette H. Chakera
author_facet Simon Naseri
Torben Steiniche
Morten Ladekarl
Marie Louise Bønnelykke-Behrndtz
Lisbet R. Hölmich
Seppo W. Langer
Alessandro Venzo
Elizaveta Tabaksblat
Siri Klausen
Mathilde Skaarup Larsen
Niels Junker
Annette H. Chakera
author_sort Simon Naseri
collection DOAJ
description 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.
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spelling doaj.art-f46deb63086a494085156fdcd46fbae32023-09-02T17:28:21ZengMDPI AGCancers2072-66942020-02-0112355410.3390/cancers12030554cancers12030554Management Recommendations for Merkel Cell Carcinoma—A Danish PerspectiveSimon Naseri0Torben Steiniche1Morten Ladekarl2Marie Louise Bønnelykke-Behrndtz3Lisbet R. Hölmich4Seppo W. Langer5Alessandro Venzo6Elizaveta Tabaksblat7Siri Klausen8Mathilde Skaarup Larsen9Niels Junker10Annette H. Chakera11Department of Pathology, Aarhus University Hospital, 8200 Aarhus, DenmarkDepartment of Pathology, Aarhus University Hospital, 8200 Aarhus, DenmarkDepartment of Oncology, Clinical Cancer Research Center, Aalborg University Hospital, 9000 Aalborg, DenmarkDepartment of Plastic and Reconstructive Surgery, Aarhus University Hospital, 8200 Aarhus, DenmarkDepartment of Plastic Surgery, Herlev & Gentofte Hospital, Department of Clinical Medicine, Copenhagen University, 2730 Herlev, DenmarkDepartment of Oncology, Copenhagen University Hospital, Rigshospitalet, 2100 Copenhagen, DenmarkDepartment of Plastic Surgery and Burns Treatment, Rigshospitalet, Copenhagen University Hospital, 2100 Copenhagen, DenmarkDepartment of Oncology, Aarhus University Hospital, 8200 Aarhus, DenmarkDepartment of Pathology, Herlev Hospital, 2730 Herlev, DenmarkDepartment of Pathology, Herlev Hospital, 2730 Herlev, DenmarkDepartment of Oncology, Herlev Hospital, 2730 Herlev, DenmarkDepartment of Plastic Surgery, Herlev & Gentofte Hospital, Department of Clinical Medicine, Copenhagen University, 2730 Herlev, DenmarkMerkel 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.https://www.mdpi.com/2072-6694/12/3/554merkel cell carcinomadiagnosistreatmentreviewguideline
spellingShingle Simon Naseri
Torben Steiniche
Morten Ladekarl
Marie Louise Bønnelykke-Behrndtz
Lisbet R. Hölmich
Seppo W. Langer
Alessandro Venzo
Elizaveta Tabaksblat
Siri Klausen
Mathilde Skaarup Larsen
Niels Junker
Annette H. Chakera
Management Recommendations for Merkel Cell Carcinoma—A Danish Perspective
Cancers
merkel cell carcinoma
diagnosis
treatment
review
guideline
title Management Recommendations for Merkel Cell Carcinoma—A Danish Perspective
title_full Management Recommendations for Merkel Cell Carcinoma—A Danish Perspective
title_fullStr Management Recommendations for Merkel Cell Carcinoma—A Danish Perspective
title_full_unstemmed Management Recommendations for Merkel Cell Carcinoma—A Danish Perspective
title_short Management Recommendations for Merkel Cell Carcinoma—A Danish Perspective
title_sort management recommendations for merkel cell carcinoma a danish perspective
topic merkel cell carcinoma
diagnosis
treatment
review
guideline
url https://www.mdpi.com/2072-6694/12/3/554
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