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PEComa—A Rare Liver Tumor

PEComa (perivascular epithelioid cell tumor) is a rare liver tumor. Decisions regarding patient management are currently based on a few small case series. The aim of this study was to report the clinicopathological features of PEComa in order to provide guidance for management, complemented by our o...

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Autores principales: Krawczyk, Marek, Ziarkiewicz-Wróblewska, Bogna, Wróblewski, Tadeusz, Podgórska, Joanna, Grzybowski, Jakub, Gierej, Beata, Krawczyk, Piotr, Nyckowski, Paweł, Kornasiewicz, Oskar, Patkowski, Waldemar, Remiszewski, Piotr, Zając, Krzysztof, Grąt, Michał
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8072725/
https://www.ncbi.nlm.nih.gov/pubmed/33919494
http://dx.doi.org/10.3390/jcm10081756
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author Krawczyk, Marek
Ziarkiewicz-Wróblewska, Bogna
Wróblewski, Tadeusz
Podgórska, Joanna
Grzybowski, Jakub
Gierej, Beata
Krawczyk, Piotr
Nyckowski, Paweł
Kornasiewicz, Oskar
Patkowski, Waldemar
Remiszewski, Piotr
Zając, Krzysztof
Grąt, Michał
author_facet Krawczyk, Marek
Ziarkiewicz-Wróblewska, Bogna
Wróblewski, Tadeusz
Podgórska, Joanna
Grzybowski, Jakub
Gierej, Beata
Krawczyk, Piotr
Nyckowski, Paweł
Kornasiewicz, Oskar
Patkowski, Waldemar
Remiszewski, Piotr
Zając, Krzysztof
Grąt, Michał
author_sort Krawczyk, Marek
collection PubMed
description PEComa (perivascular epithelioid cell tumor) is a rare liver tumor. Decisions regarding patient management are currently based on a few small case series. The aim of this study was to report the clinicopathological features of PEComa in order to provide guidance for management, complemented by our own experience. This retrospective observational study included all patients with PEComa who underwent surgical treatment in two departments between 2002 and 2020. A total of 20 patients were diagnosed with PEComa following histopathological examination. The age of the patients ranged from 21 to 73 years. The majority of patients were women (85%). In most patients, the tumors were incidental. In diagnostic studies, PEComas with high arterial vascularization have been described. Liver resection was the treatment of choice. There was only one postoperative complication. During histopathological evaluation, tumors were composed mostly of epithelioid cells, rarely with spindle cell components, thick-walled vessels, and adipocytes in different proportions. Melanocytic markers (HMB45, MelanA) and at least one smooth muscle marker were expressed in all tumors. Features suggestive of malignancy were found in three cases. In conclusion, PEComa is a rare liver tumor that is usually diagnosed incidentally. In radiological studies, tumors with high arterial vascularization are observed. Liver resection is the treatment of choice.
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spelling pubmed-80727252021-04-27 PEComa—A Rare Liver Tumor Krawczyk, Marek Ziarkiewicz-Wróblewska, Bogna Wróblewski, Tadeusz Podgórska, Joanna Grzybowski, Jakub Gierej, Beata Krawczyk, Piotr Nyckowski, Paweł Kornasiewicz, Oskar Patkowski, Waldemar Remiszewski, Piotr Zając, Krzysztof Grąt, Michał J Clin Med Article PEComa (perivascular epithelioid cell tumor) is a rare liver tumor. Decisions regarding patient management are currently based on a few small case series. The aim of this study was to report the clinicopathological features of PEComa in order to provide guidance for management, complemented by our own experience. This retrospective observational study included all patients with PEComa who underwent surgical treatment in two departments between 2002 and 2020. A total of 20 patients were diagnosed with PEComa following histopathological examination. The age of the patients ranged from 21 to 73 years. The majority of patients were women (85%). In most patients, the tumors were incidental. In diagnostic studies, PEComas with high arterial vascularization have been described. Liver resection was the treatment of choice. There was only one postoperative complication. During histopathological evaluation, tumors were composed mostly of epithelioid cells, rarely with spindle cell components, thick-walled vessels, and adipocytes in different proportions. Melanocytic markers (HMB45, MelanA) and at least one smooth muscle marker were expressed in all tumors. Features suggestive of malignancy were found in three cases. In conclusion, PEComa is a rare liver tumor that is usually diagnosed incidentally. In radiological studies, tumors with high arterial vascularization are observed. Liver resection is the treatment of choice. MDPI 2021-04-18 /pmc/articles/PMC8072725/ /pubmed/33919494 http://dx.doi.org/10.3390/jcm10081756 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/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 (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Krawczyk, Marek
Ziarkiewicz-Wróblewska, Bogna
Wróblewski, Tadeusz
Podgórska, Joanna
Grzybowski, Jakub
Gierej, Beata
Krawczyk, Piotr
Nyckowski, Paweł
Kornasiewicz, Oskar
Patkowski, Waldemar
Remiszewski, Piotr
Zając, Krzysztof
Grąt, Michał
PEComa—A Rare Liver Tumor
title PEComa—A Rare Liver Tumor
title_full PEComa—A Rare Liver Tumor
title_fullStr PEComa—A Rare Liver Tumor
title_full_unstemmed PEComa—A Rare Liver Tumor
title_short PEComa—A Rare Liver Tumor
title_sort pecoma—a rare liver tumor
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8072725/
https://www.ncbi.nlm.nih.gov/pubmed/33919494
http://dx.doi.org/10.3390/jcm10081756
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