Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome.

World J Gastroenterol

Sanya Wadhwa, Department of Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033, United States.

Published: April 2017


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Article Abstract

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.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5385411PMC
http://dx.doi.org/10.3748/wjg.v23.i13.2443DOI Listing

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