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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MDPI AG
2020-02-01
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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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issn | 2072-6694 |
language | English |
last_indexed | 2024-03-12T08:33:26Z |
publishDate | 2020-02-01 |
publisher | MDPI AG |
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series | Cancers |
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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