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Case Reports

Abstract

Angiosarcoma is a rare, aggressive cancer with reported increasing incidence. We present a 78-year-old female with a history of ductal carcinoma in situ with microinvasion treated with lumpectomy and exemestane without radiation, who presented ~8 years later with a palpable breast mass and focal edema and dermal thickening medial to the mass. There was initially clinical suspicion for inflammatory breast carcinoma; however, no malignancy was identified on skin punch biopsy. She underwent a partial mastectomy and sentinel lymph node biopsy which demonstrated an invasive ductal carcinoma (IDC) of the breast and an angiosarcoma of the skin (node-negative). The gross specimen revealed two purple-tan, ill-defined contusions in her breast skin, measuring 2.5 cm and 3 cm, respectively. Immunohistochemical (IHC) studies for the breast IDC demonstrated cytokeratin positivity with ERG and CD31 negativity; conversely, IHC for the angiosarcoma demonstrated ERG and CD31 positivity and cytokeratin negativity. MYC IHC showed increased expression in the angiosarcoma. Angiosarcoma of the breast can arise de novo (primary breast angiosarcoma and primary cutaneous angiosarcoma) or after biological insult (secondary breast angiosarcoma). Secondary angiosarcoma was first recognized after mastectomy and axillary dissection in association with chronic lymphedema (Stewart–Treves syndrome) when breast-conserving surgery was not as common. More recently, secondary angiosarcoma has been most often linked to previous radiation therapy. Interestingly, this patient received neither radiation therapy nor an axillary dissection. The purpose of this report is to describe a rare case of concurrent angiosarcoma in the breast skin and recurrent breast cancer with a unique clinical presentation.