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Primary Merkel Cell Carcinoma: A Retrospective Analysis of 31 Cases in Poland

INTRODUCTION: Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous neuroendocrine cancer that typically arises in sun-exposed areas of the skin, especially in the elderly. Significant advances have recently been made regarding skin cancers, but data on cases of MCC are rather limited as these...

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Autores principales: Ciążyńska, Magdalena, Szczepaniak, Katarzyna, Pabianek, Marta, Nejc, Dariusz, Reich, Adam, Ułańska, Małgorzata, Owczarek, Witold, Skibińska, Małgorzata, Narbutt, Joanna, Lesiak, Aleksandra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Healthcare 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7477030/
https://www.ncbi.nlm.nih.gov/pubmed/32654000
http://dx.doi.org/10.1007/s13555-020-00424-5
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author Ciążyńska, Magdalena
Szczepaniak, Katarzyna
Pabianek, Marta
Nejc, Dariusz
Reich, Adam
Ułańska, Małgorzata
Owczarek, Witold
Skibińska, Małgorzata
Narbutt, Joanna
Lesiak, Aleksandra
author_facet Ciążyńska, Magdalena
Szczepaniak, Katarzyna
Pabianek, Marta
Nejc, Dariusz
Reich, Adam
Ułańska, Małgorzata
Owczarek, Witold
Skibińska, Małgorzata
Narbutt, Joanna
Lesiak, Aleksandra
author_sort Ciążyńska, Magdalena
collection PubMed
description INTRODUCTION: Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous neuroendocrine cancer that typically arises in sun-exposed areas of the skin, especially in the elderly. Significant advances have recently been made regarding skin cancers, but data on cases of MCC are rather limited as these patients are frequently grouped together with other non-melanoma skin cancers (NMSC). Here, we performed an analysis of the clinical profile of patients with MCC in Poland to identify major factors influencing the prognosis. METHODS: Approximately 13,000 pathology and medical records were examined to identify patients with MCC diagnosed between 2010 and 2019. The management and outcomes of patients with histologically confirmed MCC were retrospectively evaluated. RESULTS: Thirty-one patients diagnosed with MCC were identified. The tumor occurred predominantly in women (61.3%) and in the elderly (mean 75.6 years). Twenty-nine patients had locoregional MCC and two had metastatic MCC at the time of diagnosis. Patients in stage I disease had excellent prognosis. In stages II and III, respectively 22.2% and 50.0% of patients developed metastases. Among patients who received chemotherapy with cisplatin and etoposide, 17% achieved partial remission with progression-free survival (PFS) of 8.0 months, and a further 50% achieved stable disease with PFS of 4.0, 4.5, and 4.5 months respectively. In 6 (19.4%) patients MCC coexisted with chronic lymphocytic leukemia (CLL). In all six cases CLL preceded MCC development. CONCLUSIONS: Female gender, tumor-free resection margins, and local disease were found to be independent prognostic factors in MCC progression. Patients with hematological malignancies, immunosuppression, and those with immune deficiencies should be closely followed up as they are predisposed to develop MCC. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1007/s13555-020-00424-5) contains supplementary material, which is available to authorized users.
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spelling pubmed-74770302020-09-18 Primary Merkel Cell Carcinoma: A Retrospective Analysis of 31 Cases in Poland Ciążyńska, Magdalena Szczepaniak, Katarzyna Pabianek, Marta Nejc, Dariusz Reich, Adam Ułańska, Małgorzata Owczarek, Witold Skibińska, Małgorzata Narbutt, Joanna Lesiak, Aleksandra Dermatol Ther (Heidelb) Original Research INTRODUCTION: Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous neuroendocrine cancer that typically arises in sun-exposed areas of the skin, especially in the elderly. Significant advances have recently been made regarding skin cancers, but data on cases of MCC are rather limited as these patients are frequently grouped together with other non-melanoma skin cancers (NMSC). Here, we performed an analysis of the clinical profile of patients with MCC in Poland to identify major factors influencing the prognosis. METHODS: Approximately 13,000 pathology and medical records were examined to identify patients with MCC diagnosed between 2010 and 2019. The management and outcomes of patients with histologically confirmed MCC were retrospectively evaluated. RESULTS: Thirty-one patients diagnosed with MCC were identified. The tumor occurred predominantly in women (61.3%) and in the elderly (mean 75.6 years). Twenty-nine patients had locoregional MCC and two had metastatic MCC at the time of diagnosis. Patients in stage I disease had excellent prognosis. In stages II and III, respectively 22.2% and 50.0% of patients developed metastases. Among patients who received chemotherapy with cisplatin and etoposide, 17% achieved partial remission with progression-free survival (PFS) of 8.0 months, and a further 50% achieved stable disease with PFS of 4.0, 4.5, and 4.5 months respectively. In 6 (19.4%) patients MCC coexisted with chronic lymphocytic leukemia (CLL). In all six cases CLL preceded MCC development. CONCLUSIONS: Female gender, tumor-free resection margins, and local disease were found to be independent prognostic factors in MCC progression. Patients with hematological malignancies, immunosuppression, and those with immune deficiencies should be closely followed up as they are predisposed to develop MCC. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1007/s13555-020-00424-5) contains supplementary material, which is available to authorized users. Springer Healthcare 2020-07-11 /pmc/articles/PMC7477030/ /pubmed/32654000 http://dx.doi.org/10.1007/s13555-020-00424-5 Text en © The Author(s) 2020 Open Access This article is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License, which permits any non-commercial use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by-nc/4.0/.
spellingShingle Original Research
Ciążyńska, Magdalena
Szczepaniak, Katarzyna
Pabianek, Marta
Nejc, Dariusz
Reich, Adam
Ułańska, Małgorzata
Owczarek, Witold
Skibińska, Małgorzata
Narbutt, Joanna
Lesiak, Aleksandra
Primary Merkel Cell Carcinoma: A Retrospective Analysis of 31 Cases in Poland
title Primary Merkel Cell Carcinoma: A Retrospective Analysis of 31 Cases in Poland
title_full Primary Merkel Cell Carcinoma: A Retrospective Analysis of 31 Cases in Poland
title_fullStr Primary Merkel Cell Carcinoma: A Retrospective Analysis of 31 Cases in Poland
title_full_unstemmed Primary Merkel Cell Carcinoma: A Retrospective Analysis of 31 Cases in Poland
title_short Primary Merkel Cell Carcinoma: A Retrospective Analysis of 31 Cases in Poland
title_sort primary merkel cell carcinoma: a retrospective analysis of 31 cases in poland
topic Original Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7477030/
https://www.ncbi.nlm.nih.gov/pubmed/32654000
http://dx.doi.org/10.1007/s13555-020-00424-5
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