Hepatic Angiosarcoma with Kasabach-Merritt Phenomenon:

A Case Report and Review of the Literature 

Autores: Fujii Fumika, Kimura Takefumi, Tanaka Naoki, Kubota Daisuke, Sugiura Ayumi, Umemura Takeji, Wada Shuichi, Tanaka Eiji

Resumen

A 76-year-old woman was referred to our hospital due to massive gingival bleeding following teeth extraction. Laboratory findings suggested disseminated intravascular coagulopathy (DIC). Enhanced computed tomography and magnetic resonance imaging disclosed multiple hypervascular liver masses of 2-6 cm in diameter, the largest of which displaying an irregular enhancement pattern. We considered that her DIC was caused by the multiple liver masses and commenced repeated erythrocyte/fresh frozen plasma infusion and gabexate mesilate administration. However, the DIC proved uncontrollable and trans-arterial embolization could not be attempted. The patient eventually died 4 months after admission due to spontaneous hepatic tumor rupture and hepatic failure. Post-mortem hepatic tumor biopsy led to a final diagnosis of hepatic angiosarcoma with Kasabach-Merritt phenomenon (KMP). Among the 7 cases of hepatic angiosarcoma representing KMP found in the literature, mortality occurred within 4 months of the appearance of bleeding tendency primarily due to abdominal bleeding and hepatic failure. The possibility of hepatic angiosarcoma should be considered in patients with DIC and hypervascular liver tumors. Since treatment is uncertain and prognosis is poor, novel diagnostic and therapeutic advances are needed for angiosarcoma.

Palabras clave: Kasabach-Merritt phenomenon DIC bleeding tendency liver angiosarcoma.

2018-06-28   |   303 visitas   |   Evalua este artículo 0 valoraciones

Vol. 17 Núm.4. Julio-Agosto 2018 Pags. 655-660 Ann Hepatol 2018; 17(4)