Angiosarcoma

 Eventually destroys hepatic parenchyma


image Solid, epithelioid, and spindled areas may be present


• Immunopositive for vascular markers: CD31, CD34, factor VIII

• Nuclear staining for transcription factors FLI-1 and ERG helps in confirming endothelial differentiation




Ancillary Tests




• High Ki-67 (> 10%), diffuse p53 and diffuse MYC staining favors angiosarcoma over benign vascular tumors


Top Differential Diagnoses




• Epithelioid hemangioendothelioma

• Carcinoma

• Other sarcomas

• Hepatic small vessel neoplasm

image
Hemorrhagic Cystic Tumor
This cross section from a partial hepatectomy for angiosarcoma shows numerous cystic, blood-filled spaces. [Courtesy C. Trower, PA (ASCP) and A. Folpe, MD.]


image
Spindled Tumor Cells
This angiosarcoma has a spindled growth pattern. The tumor cells are admixed with red blood cells and forming vascular lumina in some places. The normal hepatic architecture is no longer apparent.

image
Atypical Epithelioid Cells
Markedly atypical neoplastic endothelial cells line poorly formed vascular spaces. Note that the normal hepatic architecture has been destroyed.

image
Marked Cytologic Atypia
This angiosarcoma shows markedly atypical endothelial cells with enlarged, bizarre nuclei. The tumor cells show scattered vascular differentiation. Note the admixed red blood cells and hemosiderin-laden macrophages image .


TERMINOLOGY


Definitions




• Rare malignant vascular tumor of liver
image Most common primary hepatic sarcoma

image ∼ 2% of malignant liver tumors


ETIOLOGY/PATHOGENESIS


Environmental Exposure




• Drugs: Androgens, contraceptive steroids, diethylstilbestrol, phenelzine
• Toxins: Vinyl chloride, arsenic, copper sulfate, Thorotrast

• ∼ 25-40% of cases associated with vinyl chloride, Thorotrast, arsenic, or steroids

image Many of these agents no longer used or strictly controlled

image Long latency period of up to several decades after exposure


CLINICAL ISSUES


Epidemiology




• Age
image Peak incidence in 6th-7th decades

image Rare cases in children, formerly considered to be type 2 infantile hemangioemdothelioma

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Angiosarcoma

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