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Primary Breast Angiosarcoma : Treatment

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Treatment of primary angiosarcoma of the breast: Mastectomy is the mainstay of treatment. Axillary lymph node involvement is rare (<10% of cases) and therefore axillary node sampling is not routinely performed. The density of vascular channels and the degree of endothelial atypia often decrease at the periphery of the tumor. This makes assessment of margins by frozen section quite challenging at the time of surgery. Margins interpreted as negative on frozen section frequently turn out to be positive on permanent sections.

Metastatic angiosarcoma is treated primarily with cytotoxic chemotherapy consisting of anthracyclines, ifosfamide, and taxanes (which have anti-angiogenic activity). Newer approaches include the use of tyrosine kinase inhibitor sorafenib which targets VEGFR signaling pathways.

The image shows epithelioid angiosarcoma of breast (primary). These tumors are composed of solid sheets of large epithelioid cells with abundant eosinophilic or amphophilic cytoplasm, vesicular nuclei, and prominent nucleoli. Increased mitotic activity, hemorrhage, and necrosis are commonly present. Usually, there is no prominent vessel formation, although a few intracytoplasmic lumina with red blood cells may be seen.

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