LAUSR

Primary hepatic angiosarcoma is a rare entity, representing only 4% of all the angiosarcomas of different origins and less than 1% of all hepatic malignancies. Accurate diagnosis of this tumour is difficult, especially if the patient has no history of exposure to specific carcinogens including thorotrast, arsenicals and vinyl chloride monomer. Angiosarcoma, a subtype of soft tissue sarcoma, is an aggressive malignant disease deriving from endothelium, lymphatics or blood vessels. Some of the more common hepatic sarcomas are angiosarcoma, embryonal sarcoma, leiomyosarcoma, epithelioid hemangioendothelioma, fibrosarcoma and malignant fibrous histiocytoma. The survival of hepatic angiosarcoma is very poor, which is attributable to its rapid progress, high recurrence rate and resistant to traditional chemotherapy and radiotherapy. The survival of patients with liver angiosarcoma is very poor with median survival of 6 months without treatment; after treatment, only 3% of patients were reported to live longer than 2 years. To date, the therapeutic guideline for liver angiosarcoma has not been set up, so partial liver resection to remove tumour radically still remains to be the cornerstone of treatment options. A 72-year-old woman presented at the emergency department of our hospital with abdominal pain, fever (38.8°C), nausea and vomiting. Blood haemoglobin was 7.7 g/dL, white blood cell count was 19.500 × 10/L, platelet count was 322.000 × 10/L, the international normalized ratio was 1.52 and prothrombin activity was 51% of standard. Six months earlier, the patient had undergone abdominal ultrasound and contrast-enhanced computed tomography (CT) scan because of mild abdominal pain located in right upper quadrant. These examinations documented an 18-cm mass of the right hemiliver which presented delayed and homogeneous enhancement and features consistent with hepatic haemangioma, which was the final diagnosis. At that time, liver resection was not advised because the hemangioma had only been moderately symptomatic. Now, in the emergency department, a new abdominal CT scan documented an increase in the size of the mass (22 × 15 × 18 cm). During the arterial phase, the mass presented a peripheral enhancement; during the portal phase, the globular enhancement moved centripetally and during the delayed phase, there was a more intense peripheral enhancement with some enhanced intralesional foci. The middle and left hepatic veins were compressed and dislocated by the mass (Fig. 1). The patient was scheduled for liver resection with the presumptive diagnosis of symptomatic giant haemangioma complicated by anaemia, coagulopathy and fever. The patient underwent right hepatectomy and the post-operative course was uneventful (Figs 2,3). At pathology, the final diagnosis was primary hepatic angiosarcoma. Unlike most hepatocellular carcinomas, hepatic angiosarcoma does not arise in a background of chronic viral hepatitis. Primary Fig. 1. Abdominal computed tomography scan: huge hypodense hepatic mass which involves the right hemiliver (22 × 15 × 18 cm).