A 22-year-old Caucasian woman presented with a four month history of pain, tenderness and swelling in her right breast. Initial treatments with antibiotics and steroids were unhelpful. She had continued to have diffuse swelling and tenderness in her right breast and also noted bruising on the overlying skin. An ultrasound of the right breast showed a large, diffuse, irregular, echogenic lesion, suggesting inflammation or tumor. A mammogram confirmed diffuse mass-like density corresponding to the echogenic mass seen on the ultrasound. A bilateral breast magnetic resonance imaging (MRI) reported diffuse abnormal signal and enhancement throughout the entire right breast (Figure 1). A right breast needle biopsy was performed (Figure 2).
Correct Answer: Angiosarcoma
Primary angiosarcoma of the breast is an extremely rare tumor representing less than 1% of all soft tissue breast tumors. It arises from the connective breast tissues and may extend into the overlying skin. Angiosarcomas can arise de novo with a median age at onset of 40 years or can be secondary to radiation treatment of an epithelial breast cancer with a median onset age of 70 years with a median of 10.5 years after radiotherapy. Radiologic findings are often nonspecific and may appear completely normal in one-third of cases. The most supportive imaging exam is an MRI that can show typical malignant signs of hyperintensity on T2 images and a rapid initial intense phase followed by washout. Fine-needle aspiration or core needle biopsy is necessary to obtain a definitive diagnosis. In addition, immunohistochemistry can be useful to identify the CD31 endothelial, indicator of vascular proliferation. Other specific markers for this kind of lesions are Factor VIII, and FLI1.The prognosis is usually poor because of the high rates of local recurrence and early development of metastasis.
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