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Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome
Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagn...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Baishideng Publishing Group Inc
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5385411/ https://www.ncbi.nlm.nih.gov/pubmed/28428724 http://dx.doi.org/10.3748/wjg.v23.i13.2443 |
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author | Wadhwa, Sanya Kim, Tae Hun Lin, Leah Kanel, Gary Saito, Takeshi |
author_facet | Wadhwa, Sanya Kim, Tae Hun Lin, Leah Kanel, Gary Saito, Takeshi |
author_sort | Wadhwa, Sanya |
collection | PubMed |
description | Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagnostic criteria of disseminated intravascular coagulation. However, the mechanism that governs this coagulopathy has been poorly understood. This case report provides histological evidence of the consumption of coagulation factors along with trapped platelets occurring within the tumor, which is the foundation for the concept of Kasabach-Merritt syndrome (KMS). KMS is characterized by thrombocytopenia and hyperconsumption of coagulation factors within a vascular tumor. However, KMS associated with angiosarcoma has not been well recognized. This case report describes, for the first time, the histological evidence of KMS that occurred in an extremely rare mesenchymal malignant tumor of the liver. |
format | Online Article Text |
id | pubmed-5385411 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Baishideng Publishing Group Inc |
record_format | MEDLINE/PubMed |
spelling | pubmed-53854112017-04-20 Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome Wadhwa, Sanya Kim, Tae Hun Lin, Leah Kanel, Gary Saito, Takeshi World J Gastroenterol Case Report Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagnostic criteria of disseminated intravascular coagulation. However, the mechanism that governs this coagulopathy has been poorly understood. This case report provides histological evidence of the consumption of coagulation factors along with trapped platelets occurring within the tumor, which is the foundation for the concept of Kasabach-Merritt syndrome (KMS). KMS is characterized by thrombocytopenia and hyperconsumption of coagulation factors within a vascular tumor. However, KMS associated with angiosarcoma has not been well recognized. This case report describes, for the first time, the histological evidence of KMS that occurred in an extremely rare mesenchymal malignant tumor of the liver. Baishideng Publishing Group Inc 2017-04-07 2017-04-07 /pmc/articles/PMC5385411/ /pubmed/28428724 http://dx.doi.org/10.3748/wjg.v23.i13.2443 Text en ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved. http://creativecommons.org/licenses/by-nc/4.0/ This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. |
spellingShingle | Case Report Wadhwa, Sanya Kim, Tae Hun Lin, Leah Kanel, Gary Saito, Takeshi Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome |
title | Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome |
title_full | Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome |
title_fullStr | Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome |
title_full_unstemmed | Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome |
title_short | Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome |
title_sort | hepatic angiosarcoma with clinical and histological features of kasabach-merritt syndrome |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5385411/ https://www.ncbi.nlm.nih.gov/pubmed/28428724 http://dx.doi.org/10.3748/wjg.v23.i13.2443 |
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