Venous malformation vessels are improperly specified and hyperproliferative.

Venous malformations (VMs) are slow-flow malformations of the venous vasculature and are the most common type of vascular malformation with a prevalence of 1%. Germline and somatic mutations have been shown to contribute to VM pathogenesis, but how these mutations affect VM pathobiology is not well...

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Autores principales:	Michael J Schonning, Seung Koh, Ravi W Sun, Gresham T Richter, Andrew K Edwards, Carrie J Shawber, June K Wu
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/aeaf19c963e04891aa8fbb22eb371909
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spelling	oai:doaj.org-article:aeaf19c963e04891aa8fbb22eb3719092021-12-02T20:07:21ZVenous malformation vessels are improperly specified and hyperproliferative.1932-620310.1371/journal.pone.0252342https://doaj.org/article/aeaf19c963e04891aa8fbb22eb3719092021-01-01T00:00:00Zhttps://doi.org/10.1371/journal.pone.0252342https://doaj.org/toc/1932-6203Venous malformations (VMs) are slow-flow malformations of the venous vasculature and are the most common type of vascular malformation with a prevalence of 1%. Germline and somatic mutations have been shown to contribute to VM pathogenesis, but how these mutations affect VM pathobiology is not well understood. The goal of this study was to characterize VM endothelial and mural cell expression by performing a comprehensive expression analysis of VM vasculature. VM specimens (n = 16) were stained for pan-endothelial, arterial, venous, and endothelial progenitor cell proteins; proliferation was assessed with KI67. Endothelial cells in the VM vessels were abnormally orientated and improperly specified, as seen by the misexpression of both arterial and endothelial cell progenitor proteins not observed in control vessels. Consistent with arterialization of the endothelial cells, VM vessels were often surrounded by multiple layers of disorganized mural cells. VM endothelium also had a significant increase in proliferative endothelial cells, which may contribute to the dilated channels seen in VMs. Together the expression analysis indicates that the VM endothelium is misspecified and hyperproliferative, suggesting that VMs are biologically active lesions, consistent with clinical observations of VM progression over time.Michael J SchonningSeung KohRavi W SunGresham T RichterAndrew K EdwardsCarrie J ShawberJune K WuPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 16, Iss 5, p e0252342 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Michael J Schonning Seung Koh Ravi W Sun Gresham T Richter Andrew K Edwards Carrie J Shawber June K Wu Venous malformation vessels are improperly specified and hyperproliferative.
description	Venous malformations (VMs) are slow-flow malformations of the venous vasculature and are the most common type of vascular malformation with a prevalence of 1%. Germline and somatic mutations have been shown to contribute to VM pathogenesis, but how these mutations affect VM pathobiology is not well understood. The goal of this study was to characterize VM endothelial and mural cell expression by performing a comprehensive expression analysis of VM vasculature. VM specimens (n = 16) were stained for pan-endothelial, arterial, venous, and endothelial progenitor cell proteins; proliferation was assessed with KI67. Endothelial cells in the VM vessels were abnormally orientated and improperly specified, as seen by the misexpression of both arterial and endothelial cell progenitor proteins not observed in control vessels. Consistent with arterialization of the endothelial cells, VM vessels were often surrounded by multiple layers of disorganized mural cells. VM endothelium also had a significant increase in proliferative endothelial cells, which may contribute to the dilated channels seen in VMs. Together the expression analysis indicates that the VM endothelium is misspecified and hyperproliferative, suggesting that VMs are biologically active lesions, consistent with clinical observations of VM progression over time.
format	article
author	Michael J Schonning Seung Koh Ravi W Sun Gresham T Richter Andrew K Edwards Carrie J Shawber June K Wu
author_facet	Michael J Schonning Seung Koh Ravi W Sun Gresham T Richter Andrew K Edwards Carrie J Shawber June K Wu
author_sort	Michael J Schonning
title	Venous malformation vessels are improperly specified and hyperproliferative.
title_short	Venous malformation vessels are improperly specified and hyperproliferative.
title_full	Venous malformation vessels are improperly specified and hyperproliferative.
title_fullStr	Venous malformation vessels are improperly specified and hyperproliferative.
title_full_unstemmed	Venous malformation vessels are improperly specified and hyperproliferative.
title_sort	venous malformation vessels are improperly specified and hyperproliferative.
publisher	Public Library of Science (PLoS)
publishDate	2021
url	https://doaj.org/article/aeaf19c963e04891aa8fbb22eb371909
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Venous malformation vessels are improperly specified and hyperproliferative.

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