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

Abstract 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...

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Main Authors: Magdalena Ciążyńska, Katarzyna Szczepaniak, Marta Pabianek, Dariusz Nejc, Adam Reich, Małgorzata Ułańska, Witold Owczarek, Małgorzata Skibińska, Joanna Narbutt, Aleksandra Lesiak
Format: Article
Language:English
Published: Adis, Springer Healthcare 2020-07-01
Series:Dermatology and Therapy
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Online Access:https://doi.org/10.1007/s13555-020-00424-5
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author Magdalena Ciążyńska
Katarzyna Szczepaniak
Marta Pabianek
Dariusz Nejc
Adam Reich
Małgorzata Ułańska
Witold Owczarek
Małgorzata Skibińska
Joanna Narbutt
Aleksandra Lesiak
author_facet Magdalena Ciążyńska
Katarzyna Szczepaniak
Marta Pabianek
Dariusz Nejc
Adam Reich
Małgorzata Ułańska
Witold Owczarek
Małgorzata Skibińska
Joanna Narbutt
Aleksandra Lesiak
author_sort Magdalena Ciążyńska
collection DOAJ
description Abstract 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.
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spelling doaj.art-fd1685e6b7b847cd9ec185efe92b59792022-12-21T22:41:01ZengAdis, Springer HealthcareDermatology and Therapy2193-82102190-91722020-07-011051003101210.1007/s13555-020-00424-5Primary Merkel Cell Carcinoma: A Retrospective Analysis of 31 Cases in PolandMagdalena Ciążyńska0Katarzyna Szczepaniak1Marta Pabianek2Dariusz Nejc3Adam Reich4Małgorzata Ułańska5Witold Owczarek6Małgorzata Skibińska7Joanna Narbutt8Aleksandra Lesiak9Department of Proliferative Diseases, Nicolaus Copernicus Multidisciplinary Centre for Oncology and TraumatologyDepartment of Proliferative Diseases, Nicolaus Copernicus Multidisciplinary Centre for Oncology and TraumatologyDepartment of Proliferative Diseases, Nicolaus Copernicus Multidisciplinary Centre for Oncology and TraumatologyDepartment of Surgical Oncology Chair of Oncology, Medical University in Łódź, Nicolaus Copernicus Multidisciplinary Centre for Oncology and TraumatologyDepartment of Dermatology, University of RzeszowDepartment of Proliferative Diseases, Nicolaus Copernicus Multidisciplinary Centre for Oncology and TraumatologyDermatology Clinic, Military Institute of Medicine in WarsawDepartment of Dermatology, Paediatric Dermatology and Oncology Clinic, Medical University of ŁódźDepartment of Dermatology, Paediatric Dermatology and Oncology Clinic, Medical University of ŁódźDepartment of Dermatology, Paediatric Dermatology and Oncology Clinic, Medical University of ŁódźAbstract 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.https://doi.org/10.1007/s13555-020-00424-5ChemotherapyMerkel cell carcinomaSentinel lymph node biopsy
spellingShingle Magdalena Ciążyńska
Katarzyna Szczepaniak
Marta Pabianek
Dariusz Nejc
Adam Reich
Małgorzata Ułańska
Witold Owczarek
Małgorzata Skibińska
Joanna Narbutt
Aleksandra Lesiak
Primary Merkel Cell Carcinoma: A Retrospective Analysis of 31 Cases in Poland
Dermatology and Therapy
Chemotherapy
Merkel cell carcinoma
Sentinel lymph node biopsy
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 Chemotherapy
Merkel cell carcinoma
Sentinel lymph node biopsy
url https://doi.org/10.1007/s13555-020-00424-5
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