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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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Autores principales: Naseri, Simon, Steiniche, Torben, Ladekarl, Morten, Bønnelykke-Behrndtz, Marie Louise, R. Hölmich, Lisbet, W. Langer, Seppo, Venzo, Alessandro, Tabaksblat, Elizaveta, Klausen, Siri, Skaarup Larsen, Mathilde, Junker, Niels, Chakera, Annette H.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7139291/
https://www.ncbi.nlm.nih.gov/pubmed/32121063
http://dx.doi.org/10.3390/cancers12030554
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author Naseri, Simon
Steiniche, Torben
Ladekarl, Morten
Bønnelykke-Behrndtz, Marie Louise
R. Hölmich, Lisbet
W. Langer, Seppo
Venzo, Alessandro
Tabaksblat, Elizaveta
Klausen, Siri
Skaarup Larsen, Mathilde
Junker, Niels
Chakera, Annette H.
author_facet Naseri, Simon
Steiniche, Torben
Ladekarl, Morten
Bønnelykke-Behrndtz, Marie Louise
R. Hölmich, Lisbet
W. Langer, Seppo
Venzo, Alessandro
Tabaksblat, Elizaveta
Klausen, Siri
Skaarup Larsen, Mathilde
Junker, Niels
Chakera, Annette H.
author_sort Naseri, Simon
collection PubMed
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 pubmed-71392912020-04-10 Management Recommendations for Merkel Cell Carcinoma—A Danish Perspective Naseri, Simon Steiniche, Torben Ladekarl, Morten Bønnelykke-Behrndtz, Marie Louise R. Hölmich, Lisbet W. Langer, Seppo Venzo, Alessandro Tabaksblat, Elizaveta Klausen, Siri Skaarup Larsen, Mathilde Junker, Niels Chakera, Annette H. Cancers (Basel) Review 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. MDPI 2020-02-28 /pmc/articles/PMC7139291/ /pubmed/32121063 http://dx.doi.org/10.3390/cancers12030554 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Naseri, Simon
Steiniche, Torben
Ladekarl, Morten
Bønnelykke-Behrndtz, Marie Louise
R. Hölmich, Lisbet
W. Langer, Seppo
Venzo, Alessandro
Tabaksblat, Elizaveta
Klausen, Siri
Skaarup Larsen, Mathilde
Junker, Niels
Chakera, Annette H.
Management Recommendations for Merkel Cell Carcinoma—A Danish Perspective
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 Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7139291/
https://www.ncbi.nlm.nih.gov/pubmed/32121063
http://dx.doi.org/10.3390/cancers12030554
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