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WHO Classification of Tumours
Angiosarcoma
Liver and intrahepatic bile ducts


Definition

A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.

Hepatic angiosarcoma
The typical occurrence is in patients aged > 60 years and there is a 3 : 1 predominance in men. However, angiosarcomas occur even in children with a very low frequency. Known etiological factors (seen in 20% of hepatic angiosarcomas) include occupational exposure to vinyl chloride monomer, arsenic, and androgenic-anabolic steroids. Iatrogenic thorium oxide (thorothrast), an α particle-radiating substance previously used as a radiological contrast medium, is historically important, but presently a very rare etiological factor. Prognosis is poor, and most patients die within 1 year.

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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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