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Hepatic angiosarcoma with clinical and histological features of kasabach-merritt syndrome

World Journal of Gastroenterology 2017年第13期23卷 2443-2447页

作者： Sanya Wadhwa Tae Hun Kim Leah Lin Gary Kanel Takeshi Saito Department of Medicine Keck School of MedicineUniversity of Southern California Department of Pathology University of Southern CaliforniaKeck School of Medicine Department of Radiology University of Southern CaliforniaKeck School of Medicine Department of Medicine Molecular Microbiology and Immunologyand PathologyDivision of Gastrointestinal and Liver DiseasesKeck School of Medicine of USCUniversity of Southern California

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.

关键词： Hepatic angiosarcoma kasabach-merritt syndrome Vascular tumor

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