[Primary angiosarcoma of the liver].

Hepatic angiosarcoma (AS) is a rare tumor with a fatal course. It represents 1-2% of all primary hepatic tumors and it is the most frequent primary mesenchymal tumor of the liver. Recently its incidence is dramatically increased because of its epidemiological association with exposure to thorotrast, vinyl chloride and other carcinogens. We present the epidemiological, clinical, diagnostic and therapeutic features of 6 patients with primary hepatic AS. No apparent predisposing factors were present. At presentation symptoms, signs and liver function tests were not specific. Four patients presented with metastatic disease: lung (2), peritoneum (1), bone and spleen (1). At time of diagnosis tumor was bilobar in 4 cases and in 2 of whom was multifocal. Median tumor size was 10.5 cm (range: 4-20 cm). Four patients with extrahepatic disease were conservatively treated with chemotherapy (VAC) and the longest survival was 8 months and 2 with tumor confined to the liver were transplanted but both had an early recurrence at 3 and 5 months post-tx respectively and the longest survival was 10 months. These disappointing results confirm the aggressive nature of hepatic AS. An early diagnosis is needed and the radical resection of the tumor is still the best form of treatment.