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Vol 59, No 4 (2021)
Original paper
Submitted: 2021-05-28
Accepted: 2021-11-30
Published online: 2021-12-08
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New insights into the pathogenesis of cardiac papillary fibroelastomas

Natalia Matysiak1, Lukasz Mielanczyk1, Krzysztof Kaczmarek2, Malgorzata Zaba1, Edyta Reichman-Warmusz1, Romuald Wojnicz1
DOI: 10.5603/FHC.a2021.0027
·
Pubmed: 34878643
·
Folia Histochem Cytobiol 2021;59(4):212-225.
Affiliations
  1. Department of Histology and Cell Pathology, FMS in Zabrze, Medical University of Silesia, Katowice, Poland
  2. Department of Electrocardiology, Medical University of Łodź, Łodź, Poland

open access

Vol 59, No 4 (2021)
ORIGINAL PAPERS
Submitted: 2021-05-28
Accepted: 2021-11-30
Published online: 2021-12-08

Abstract

Introduction. Cardiac papillary fibroelastomas (CPFs) are rare benign cardiac tumors typically found on the heart valves. The previously published data on the CPF focused on its clinical presentation, optimal management, and prognosis. However, histogenesis of these lesions remains controversial. Accordingly, the aim of this study was to establish the role of endocardial endothelium (EE) in CPF formation.

Materials and methods. Four CPF tumors removed from the right atrioventricular valves were analyzed using hematoxylin & eosin, orcein, and Masson trichrome staining together with immunochemistry for CD-34, CD-68, vimentin, vWF and a-SMA. Moreover, conventional transmission electron microscopy was used for morphological analysis and a-SMA presence confirmation.

Results. Ultrastructural morphology, immunohisto- and immunocytochemical analyses indicated that cells covering collagenous core have an endothelial origin. Some endocardial endothelium cells have the potential to undergo a transition to mesenchymal cells. Moreover, the abundant presence of extracellular vesicles may indicate an active intercellular communication. Within the intermediate translucent zone, amorphous substances with monocytes/macrophage-like cells and fibroblastic cells were found. Finally, within collagenous core activated (myo)fibroblasts were observed.

Conclusions. Our study demonstrated that the endocardial endothelium of the CPF was “double-sided”, i.e., it presented both endothelial and mesenchymal cell characteristics. Another finding was the presence of monocytes, and macrophages which were integrated into CPF core and displayed features of a fibroblast that have been shown to contribute to extracellular matrix production. This could be interpreted as being attributed to the CPF histogenesis.

Abstract

Introduction. Cardiac papillary fibroelastomas (CPFs) are rare benign cardiac tumors typically found on the heart valves. The previously published data on the CPF focused on its clinical presentation, optimal management, and prognosis. However, histogenesis of these lesions remains controversial. Accordingly, the aim of this study was to establish the role of endocardial endothelium (EE) in CPF formation.

Materials and methods. Four CPF tumors removed from the right atrioventricular valves were analyzed using hematoxylin & eosin, orcein, and Masson trichrome staining together with immunochemistry for CD-34, CD-68, vimentin, vWF and a-SMA. Moreover, conventional transmission electron microscopy was used for morphological analysis and a-SMA presence confirmation.

Results. Ultrastructural morphology, immunohisto- and immunocytochemical analyses indicated that cells covering collagenous core have an endothelial origin. Some endocardial endothelium cells have the potential to undergo a transition to mesenchymal cells. Moreover, the abundant presence of extracellular vesicles may indicate an active intercellular communication. Within the intermediate translucent zone, amorphous substances with monocytes/macrophage-like cells and fibroblastic cells were found. Finally, within collagenous core activated (myo)fibroblasts were observed.

Conclusions. Our study demonstrated that the endocardial endothelium of the CPF was “double-sided”, i.e., it presented both endothelial and mesenchymal cell characteristics. Another finding was the presence of monocytes, and macrophages which were integrated into CPF core and displayed features of a fibroblast that have been shown to contribute to extracellular matrix production. This could be interpreted as being attributed to the CPF histogenesis.

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Keywords

cardiac papillary fibroelastoma; endocardial endothelium; endothelial to mesenchymal transition; EMT; ectosomes; myofibroblast; cardiac benign tumors; electron microscopy

About this article
Title

New insights into the pathogenesis of cardiac papillary fibroelastomas

Journal

Folia Histochemica et Cytobiologica

Issue

Vol 59, No 4 (2021)

Article type

Original paper

Pages

212-225

Published online

2021-12-08

Page views

6586

Article views/downloads

585

DOI

10.5603/FHC.a2021.0027

Pubmed

34878643

Bibliographic record

Folia Histochem Cytobiol 2021;59(4):212-225.

Keywords

cardiac papillary fibroelastoma
endocardial endothelium
endothelial to mesenchymal transition
EMT
ectosomes
myofibroblast
cardiac benign tumors
electron microscopy

Authors

Natalia Matysiak
Lukasz Mielanczyk
Krzysztof Kaczmarek
Malgorzata Zaba
Edyta Reichman-Warmusz
Romuald Wojnicz

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