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Primary Breast Angiosarcoma : Clinical & Gross

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Angiosarcomas of the breast occur in two clinical settings: 1) as primary angiosarcomas; or 2) following surgical and/or radiation treatment for breast cancer. The sites of involvement include breast parenchyma proper, skin and subcutaneous tissues of breast, chest wall post-mastectomy, and upper extremity post-mastectomy.

Primary angiosarcoma of breast, by definition, arises in the parenchyma of untreated breast (i.e. no history of surgery and/or radiation). It accounts for less than 0.1% of all primary malignancies of the breast. It occurs over a wide age range; however, the median age is 3rd or 4th decade of life. Rare cases have been reported in men.

Clinical Features: They present as an ill-defined breast mass about 5-7 cm in size with red, purple, or bluish mottling of the overlying skin. Some patients have a painless diffuse enlargement of their breast without a discrete mass. The vast majority of cases are localized to breast at presentation. Nipple retraction is not a feature.

Gross Pathology: The tumor ranges from 3-4 cm to as much as 25 cm in size. The microscopic extent of the tumor is usually greater than gross size would indicate. There is no correlation between tumor size and histologic grade. Grossly, they are deep-seated, ill-defined, spongy, hemorrhagic lesions. High-grade lesions may appear as ill-defined areas of induration without any obvious vascular component.

This mastectomy specimen from a 58 y/o female shows several bluish purple nodules under the skin. They were scattered throughout the breast parenchyma and had a hemorrhagic appearance (see next image). Microscopic examination confirmed the diagnosis of primary angiosarcoma of the breast, low-grade.

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