Merkel cell carcinoma

Abstract Merkel cell carcinoma (MCC) is a rare neuroendocrine carcinoma. The cellular origin of MCC may include Merkel cell precursors. The incidence of MCC has increased significantly however trends may have been confounded by evolving diagnostic criteria. The two key aetiologies of MCC are ultravi...

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Autores principales:	K. Mistry, N. J. Levell, P. Craig, N. M. Steven, Z. C. Venables
Formato:	article
Lenguaje:	EN
Publicado:	Wiley 2021
Materias:	Dermatology RL1-803
Acceso en línea:	https://doaj.org/article/1dfa3e84db2c40d89081195d2aee527b
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spelling	oai:doaj.org-article:1dfa3e84db2c40d89081195d2aee527b2021-12-02T11:13:08ZMerkel cell carcinoma2690-442X10.1002/ski2.55https://doaj.org/article/1dfa3e84db2c40d89081195d2aee527b2021-12-01T00:00:00Zhttps://doi.org/10.1002/ski2.55https://doaj.org/toc/2690-442XAbstract Merkel cell carcinoma (MCC) is a rare neuroendocrine carcinoma. The cellular origin of MCC may include Merkel cell precursors. The incidence of MCC has increased significantly however trends may have been confounded by evolving diagnostic criteria. The two key aetiologies of MCC are ultraviolet radiation and Merkel cell polyoma virus (MCPyV). Both have unique mechanisms of carcinogenesis. MCC presents non‐specifically as a rapidly growing, red‐to‐violet nodule on sun‐exposed areas. Diagnostic accuracy has improved through immunohistochemical markers such as CK‐20. Lymph nodes should be evaluated in MCC through examination and sentinel biopsy. USS, CT, MRI and CT‐PET may be useful in staging. Management depends on tumour location, stage and comorbidities. MCPyV status may guide treatment strategy in the future. Treatment for the primary MCC is commonly wide local excision followed by radiotherapy, guided by anatomical constraints. There is uncertainty about surgical margins. Treatments for nodal disease have not been determined through trials. They include nodal dissection or radiotherapy for clinically or radiologically apparent disease, and adjuvant nodal irradiation for negative nodes, microscopic disease or following nodal dissection for definite disease. Patients with loco‐regional advanced inoperable disease should be considered for combination therapy including chemotherapy, radiotherapy, surgery and immunotherapy. Systemic therapy for advanced disease includes immune checkpoint inhibitors targeting the PD‐1/PD‐L1 pathway. Avelumab can improve survival in metastatic MCC. Immunotherapy may result in longer disease control. Various other immunotherapeutic and molecular agents are undergoing trials. MCC continues to have a high mortality characterized by high recurrence and early metastases.K. MistryN. J. LevellP. CraigN. M. StevenZ. C. VenablesWileyarticleDermatologyRL1-803ENSkin Health and Disease, Vol 1, Iss 4, Pp n/a-n/a (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Dermatology RL1-803
spellingShingle	Dermatology RL1-803 K. Mistry N. J. Levell P. Craig N. M. Steven Z. C. Venables Merkel cell carcinoma
description	Abstract Merkel cell carcinoma (MCC) is a rare neuroendocrine carcinoma. The cellular origin of MCC may include Merkel cell precursors. The incidence of MCC has increased significantly however trends may have been confounded by evolving diagnostic criteria. The two key aetiologies of MCC are ultraviolet radiation and Merkel cell polyoma virus (MCPyV). Both have unique mechanisms of carcinogenesis. MCC presents non‐specifically as a rapidly growing, red‐to‐violet nodule on sun‐exposed areas. Diagnostic accuracy has improved through immunohistochemical markers such as CK‐20. Lymph nodes should be evaluated in MCC through examination and sentinel biopsy. USS, CT, MRI and CT‐PET may be useful in staging. Management depends on tumour location, stage and comorbidities. MCPyV status may guide treatment strategy in the future. Treatment for the primary MCC is commonly wide local excision followed by radiotherapy, guided by anatomical constraints. There is uncertainty about surgical margins. Treatments for nodal disease have not been determined through trials. They include nodal dissection or radiotherapy for clinically or radiologically apparent disease, and adjuvant nodal irradiation for negative nodes, microscopic disease or following nodal dissection for definite disease. Patients with loco‐regional advanced inoperable disease should be considered for combination therapy including chemotherapy, radiotherapy, surgery and immunotherapy. Systemic therapy for advanced disease includes immune checkpoint inhibitors targeting the PD‐1/PD‐L1 pathway. Avelumab can improve survival in metastatic MCC. Immunotherapy may result in longer disease control. Various other immunotherapeutic and molecular agents are undergoing trials. MCC continues to have a high mortality characterized by high recurrence and early metastases.
format	article
author	K. Mistry N. J. Levell P. Craig N. M. Steven Z. C. Venables
author_facet	K. Mistry N. J. Levell P. Craig N. M. Steven Z. C. Venables
author_sort	K. Mistry
title	Merkel cell carcinoma
title_short	Merkel cell carcinoma
title_full	Merkel cell carcinoma
title_fullStr	Merkel cell carcinoma
title_full_unstemmed	Merkel cell carcinoma
title_sort	merkel cell carcinoma
publisher	Wiley
publishDate	2021
url	https://doaj.org/article/1dfa3e84db2c40d89081195d2aee527b
work_keys_str_mv	AT kmistry merkelcellcarcinoma AT njlevell merkelcellcarcinoma AT pcraig merkelcellcarcinoma AT nmsteven merkelcellcarcinoma AT zcvenables merkelcellcarcinoma
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Merkel cell carcinoma

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