Primary Hepatic Angiosarcoma: A Brief Review of the Literature

Abstract

Primary hepatic angiosarcoma (HAS) is a liver tumour of endothelial cell origin. It is the most common malignant mesenchymal tumour of the liver, but is nonetheless rare, accounting for approximately 0.1–2.0% of all primary liver malignancies. Historically, 25% of HAS cases were associated with occupational or medicinal exposure, but most cases are now considered idiopathic. Patients present with vague signs and symptoms of liver disease, often resulting in late diagnoses; patients may present with acute liver failure or spontaneous rupture of the tumour, but this is rare. Preoperative diagnosis of HAS is difficult because laboratory and radiological findings are often non-specific or unable to discern malignant masses from benign growths. Obtaining a biopsy for histopathological diagnosis of HAS is also difficult because of its vascular and haemorrhagic nature, and reports of death from closed biopsies have been noted. Prognosis is poor because of the disease’s diagnostic challenges and the tumour’s rapidly progressive and early metastatic nature. The reported median survival is approximately 6 months, with only 3% of patients living >2 years. This paper will review and summarise new and existing publications in the English language literature to provide a better understanding of the challenges posed by HAS.