Endothelial cell malignancies: new insights from the laboratory and clinic

Abstract Endothelial cell malignancies are rare in the Western world and range from intermediate grade hemangioendothelioma to Kaposi sarcoma to aggressive high-grade angiosarcoma that metastasize early and have a high rate of mortality. These malignancies are associated with dysregulation of normal...

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Autores principales: Michael J. Wagner, Vinod Ravi, David G. Menter, Anil K. Sood
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Publicado: Nature Portfolio 2017
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spelling oai:doaj.org-article:156ffe4469384bac8a0ce449045146962021-12-02T14:18:30ZEndothelial cell malignancies: new insights from the laboratory and clinic10.1038/s41698-017-0013-22397-768Xhttps://doaj.org/article/156ffe4469384bac8a0ce449045146962017-04-01T00:00:00Zhttps://doi.org/10.1038/s41698-017-0013-2https://doaj.org/toc/2397-768XAbstract Endothelial cell malignancies are rare in the Western world and range from intermediate grade hemangioendothelioma to Kaposi sarcoma to aggressive high-grade angiosarcoma that metastasize early and have a high rate of mortality. These malignancies are associated with dysregulation of normal endothelial cell signaling pathways, including the vascular endothelial growth factor, angiopoietin, and Notch pathways. Discoveries over the past two decades related to mechanisms of angiogenesis have led to the development of many drugs that intuitively would be promising therapeutic candidates for these endothelial-derived tumors. However, clinical efficacy of such drugs has been limited. New insights into the mechanisms that lead to dysregulated angiogenesis such as mutation or amplification in known angiogenesis related genes, viral infection, and chromosomal translocations have improved our understanding of the pathogenesis of endothelial malignancies and how they evade anti-angiogenesis drugs. In this review, we describe the major molecular alterations in endothelial cell malignancies and consider emerging opportunities for improving therapeutic efficacy against these rare but deadly tumors.Michael J. WagnerVinod RaviDavid G. MenterAnil K. SoodNature PortfolioarticleNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENnpj Precision Oncology, Vol 1, Iss 1, Pp 1-13 (2017)
institution DOAJ
collection DOAJ
language EN
topic Neoplasms. Tumors. Oncology. Including cancer and carcinogens
RC254-282
spellingShingle Neoplasms. Tumors. Oncology. Including cancer and carcinogens
RC254-282
Michael J. Wagner
Vinod Ravi
David G. Menter
Anil K. Sood
Endothelial cell malignancies: new insights from the laboratory and clinic
description Abstract Endothelial cell malignancies are rare in the Western world and range from intermediate grade hemangioendothelioma to Kaposi sarcoma to aggressive high-grade angiosarcoma that metastasize early and have a high rate of mortality. These malignancies are associated with dysregulation of normal endothelial cell signaling pathways, including the vascular endothelial growth factor, angiopoietin, and Notch pathways. Discoveries over the past two decades related to mechanisms of angiogenesis have led to the development of many drugs that intuitively would be promising therapeutic candidates for these endothelial-derived tumors. However, clinical efficacy of such drugs has been limited. New insights into the mechanisms that lead to dysregulated angiogenesis such as mutation or amplification in known angiogenesis related genes, viral infection, and chromosomal translocations have improved our understanding of the pathogenesis of endothelial malignancies and how they evade anti-angiogenesis drugs. In this review, we describe the major molecular alterations in endothelial cell malignancies and consider emerging opportunities for improving therapeutic efficacy against these rare but deadly tumors.
format article
author Michael J. Wagner
Vinod Ravi
David G. Menter
Anil K. Sood
author_facet Michael J. Wagner
Vinod Ravi
David G. Menter
Anil K. Sood
author_sort Michael J. Wagner
title Endothelial cell malignancies: new insights from the laboratory and clinic
title_short Endothelial cell malignancies: new insights from the laboratory and clinic
title_full Endothelial cell malignancies: new insights from the laboratory and clinic
title_fullStr Endothelial cell malignancies: new insights from the laboratory and clinic
title_full_unstemmed Endothelial cell malignancies: new insights from the laboratory and clinic
title_sort endothelial cell malignancies: new insights from the laboratory and clinic
publisher Nature Portfolio
publishDate 2017
url https://doaj.org/article/156ffe4469384bac8a0ce44904514696
work_keys_str_mv AT michaeljwagner endothelialcellmalignanciesnewinsightsfromthelaboratoryandclinic
AT vinodravi endothelialcellmalignanciesnewinsightsfromthelaboratoryandclinic
AT davidgmenter endothelialcellmalignanciesnewinsightsfromthelaboratoryandclinic
AT anilksood endothelialcellmalignanciesnewinsightsfromthelaboratoryandclinic
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