Eventually destroys hepatic parenchyma
Solid, epithelioid, and spindled areas may be present
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Immunopositive for vascular markers: CD31, CD34, factor VIII
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Nuclear staining for transcription factors FLI-1 and ERG helps in confirming endothelial differentiation
Ancillary Tests
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High Ki-67 (> 10%), diffuse p53 and diffuse MYC staining favors angiosarcoma over benign vascular tumors
Top Differential Diagnoses
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Epithelioid hemangioendothelioma
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Hepatic small vessel neoplasm
TERMINOLOGY
Definitions
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Rare malignant vascular tumor of liver
Most common primary hepatic sarcoma
∼ 2% of malignant liver tumors
ETIOLOGY/PATHOGENESIS
Environmental Exposure
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Drugs: Androgens, contraceptive steroids, diethylstilbestrol, phenelzine
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Toxins: Vinyl chloride, arsenic, copper sulfate, Thorotrast
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∼ 25-40% of cases associated with vinyl chloride, Thorotrast, arsenic, or steroids
Many of these agents no longer used or strictly controlled
Long latency period of up to several decades after exposure
CLINICAL ISSUES
Epidemiology