Angiosarcoma

A malignant tumour composed of spindle or pleomorphic cells that line, or grow into, the lumina of preexisting vascular spaces, such as liver sinusoids and small veins.

Worldwide, about 200 cases of angiosar-coma are diagnosed annually {848, 59}. During the period 1973-87, the SEER database of the US National Cancer Institute contained 6,391 histologically-confirmed primary liver cancers; of these only 65 (1%) were angiosarcomas {252}. The peak incidence is in the 6th and 7th decades of life. The male to female ratio is 3:1 {1085}.

75% of angiosarcomas of the liver have no known aetiology {484}. The remainder have been linked to prior administration of Thorotrast (a radioactive material containing thorium dioxide, that was used as an angiography contrast medium from the 1930s to the early 1950s), exposure to vinyl chloride monomer (VCM) or inorganic arsenic, and the use of andro-genic-anabolic steroids {484}. Patients with angiosarcoma present in one of several ways: 61% have symptoms referable to the liver (e.g. hepatomegaly, abdominal pain, ascites); 15% have an acute abdominal crisis due to haemoperitoneum from rupture of the tumour; 15% have splenomegaly, often with pancytopenia; and 9% present due to distant metastases {804}. The prognosis of angiosarcoma is very poor, with most patients dying within 6 months of diagnosis.

Macroscopy. Angiosarcoma typically affects the entire liver. Grayish-white

Fig. 8.71 Angiosarcoma. A Multiple dark brown tumour foci scattered throughout the liver. B Solid portion showing spindle cells and numerous small vascular channels. C Intravascular papillary structure covered by neoplastic endothelial cells. D Tumour cells express CD34.

Fig. 8.71 Angiosarcoma. A Multiple dark brown tumour foci scattered throughout the liver. B Solid portion showing spindle cells and numerous small vascular channels. C Intravascular papillary structure covered by neoplastic endothelial cells. D Tumour cells express CD34.

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