Clinical manifestations of primary hepatic angiosarcoma

Malignant tumors of the liver stemming from mesenchymal origins are rare neoplasms, < 1% of primary malignant hepatic lesions. Primary hepatic angiosarcoma (PHA) is the most common (36%). This study describes the incidence and clinical characteristics of this rare tumor in two medical centers, over the past 18 years. We reviewed tumor registry files at Jackson Memorial Hospital and oncology data records at Cedar's Medical Center, 1979-1997. A total of 865 primary hepatic tumors were identified, of which five cases (0.58%) were PHA; four were men, and the median age was 53 years. Symptoms and signs included: pain, anemia, fever of unknown origin, weight loss, abdominal mass, and hemoperitoneum. Median survival was only 6 months. In conclusion, primary hepatic angiosarcomas frequently are symptomatic. The presentation and preexisting factors are valuable in establishing a clinical suspicion to diagnose this rare tumor. Although imaging studies are helpful, they are not conclusive, and liver biopsy is usually required.