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

Abstract

Background. Hepatic angiosarcoma is an uncommon, malignant, primary liver tumor, comprising 2% of liver cancers and accounting for < 1% of all sarcomas. Patients usually present with nonspecific symptoms, such as fatigue, weight loss, right upper quadrant pain, anemia, which leads to late diagnosis of an advanced stage tumor. The median life expectancy after the diagnosis of hepatic angiosarcoma is about 6 months, with only 3% of patients surviving more than 2 years. Liver failure and hemoperitoneum are the leading causes of death in patients with liver angiosarcoma. In rarer cases, it might cause paraneoplastic syndromes such as disseminated intravascular coagulopathy. The treatment of angiosarcomas is complicated as there are no established and effective treatment guidelines due to the tumor’s low frequency and aggressive nature.Case summary. We present the case of a 68-year old woman who was admitted to the hospital due to fatigueand severe anemia (hemoglobin 65 g/l). Laboratory results also revealed high-grade thrombocytopenia(8 × 109/l). The abdominal ultrasound and computed tomography scan showed multiple lesions throughout with hepatic angiosarcoma. The treatment with first-line chemotherapy (doxorubicin) was initiated despiteongoing paraneoplastic syndrome – disseminative intravascular coagulopathy. However, the disease was terminal, and the patient died 2 months since diagnosed.Conclusions. Hepatic angiosarcoma is a rare and terminal tumor. Therefore, knowledge about its manifestations and effective treatment methods is lacking. Disseminative intravascular coagulopathy is a unique clinical characteristic of angiosarcoma seen in a subset of patients.

Primary hepatic angiosarcoma: A case report

“…Studies have reported that PHA is closely related to D-dimer and platelet levels, manifesting as increased D-dimer levels and thrombocytopenia. Physiological conditions caused by malignant endodermic cells can promote platelet adhesion and activation, leading to the excessive consumption of platelets and coagulation factors at the tumor site[ 6 , 7 ].…”

Rare infiltrative primary hepatic angiosarcoma: A case report and review of literature

“…PHA progresses rapidly, with extrahepatic metastasis occurring in half of patients at the time of discovery, the most common sites of metastasis in PHA are the spleen, lungs and bone[ 16 ]. Intrasplenic metastasis are rarer, so it provides favorable diagnostic clues for the diagnosis of PHA[ 17 - 19 ].…”