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Presented by Dr. Ezra Baraban and prepared by Dr. Sintawat Wangsiricharoen.
A 60-year-old man with a thigh mass.
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Answer: B. Epithelioid angiosarcoma.
Sections show a high grade malignant epithelioid neoplasm with focal prominent intercellular hemorrhage. The differential diagnosis is broad, and includes sarcomatoid carcinoma, melanoma, and various sarcomas. Negativity for S100 and SOX10 eliminate melanoma. Lack of primary carcinoma elsewhere and the absence of an epithelial component argue against sarcomatoid carcinoma. Expression of ERG, CD31, and CD34 support epithelioid angiosarcoma. Importantly, epithelioid sarcoma may express multiple endothelial markers, particularly CD34 and ERG. CD31 is typically negative, and importantly, INI1 expression will invariably be absent in epithelioid sarcoma and retained in epithelioid angiosarcoma.
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Answer: B. Epithelioid angiosarcoma.
Sections show a high grade malignant epithelioid neoplasm with focal prominent intercellular hemorrhage. The differential diagnosis is broad, and includes sarcomatoid carcinoma, melanoma, and various sarcomas. Negativity for S100 and SOX10 eliminate melanoma. Lack of primary carcinoma elsewhere and the absence of an epithelial component argue against sarcomatoid carcinoma. Expression of ERG, CD31, and CD34 support epithelioid angiosarcoma. Importantly, epithelioid sarcoma may express multiple endothelial markers, particularly CD34 and ERG. CD31 is typically negative, and importantly, INI1 expression will invariably be absent in epithelioid sarcoma and retained in epithelioid angiosarcoma.