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

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.

Primary Hepatic Angiosarcoma Revealed by a Hemorrhagic Parietal Metastasis about a Case in Lomé

“…Similarly, the chemotherapy initiated since histological confirmation did not lead to a significant improvement in the clinical status of the patient, in correlation with the data found in the literature [1] [8] [9]. The median survival is 5 months [10], however, in our case history taking, the patient survived six months.…”

Diffused hepatic angiosarcoma with Kasabach-Merritt syndrome-case report and literature review

“…Fig. 1 Abdominal computed tomography showed enlarged liver, irregular liver contours, heterogeneous hepatic parenchyma with diffusely distributed multiple hypodense focal lesions Despite the hypothesis that KMP is exclusively limited to these two vascular formations in paediatric populations [11], there have been several case reports about hepatic angiosarcoma with KMS in adults recently [12]. KMS is characterized by anemia, thrombocytopenia, prolonged prothrombin time, hypofibrinogenemia, and obviously increased fibrinogen degradation product and D-dimer.…”

Paraneoplastic Phenomena of Disseminated Intravascular Coagulopathy in Hepatic Angiosarcoma – Rare, Challenging and Fatal. Case Report and Literature Review

“…HA most commonly spreads to the lungs, spleen and bones [11][12][13]. In a subset of patients, the pronounced dysregulation of the coagulation system is detected, also known as disseminated intravascular coagulation (DIC), which is mostly indistinguishable from Kasabach-Merritt syndrome (KMS) [14].…”