Published online Apr 7, 2017. doi: 10.3748/wjg.v23.i13.2443
Peer-review started: October 30, 2016
First decision: December 19, 2016
Revised: December 23, 2016
Accepted: January 4, 2017
Article in press: January 4, 2017
Published online: April 7, 2017
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.
Core tip: Kasabach-Merritt syndrome (KMS) is characterized by thrombocytopenia and hyperconsumption of coagulation factors within a vascular tumor. KMS is typically seen in the pediatric population however there have been reports of KMS occurring in association with adult vascular tumors. Based on laboratory findings, it is hard to differentiate KMS from disseminated intravascular coagulation. Here, we describe, for the first time the histological evidence validating the concept of KMS.