Primary and secondary angiosarcoma of the breast

Abstract

Angiosarcoma is a rare soft tissue tumor of the breast. It occurs in both a primary form without a known precursor, and a secondary form that has been associated to a history of irradiated breast tissue. These forms differ in many ways including median age, precipitating factors, and presentation. Both forms have a malignant behavior and a poor prognosis. The endeavor of this paper is to review what is known about the presentation, diagnostic and therapeutic modalities to date.

Keywords: Breast; aggressive; cancer; radiation; rapid growth.

Figure 1

(A) Macroscopic appearance of the right breast affected by secondary angiosarcoma eight years after adjuvant radiation. Note enlargement and discoloration of the right breast; (B) Advanced disease with discolored nodular lesions; (C) Local recurrence three months after complete removal with negative margins.

Figure 2

(A) Mammary angiosarcoma (10×) demonstrating vascular channels dissecting through connective tissue and forming anastomotic networks; (B) Higher power showing cytologically atypical endothelial cells with high N:C ratio and large hyperchromatic nuclei (40×); (C) Tumor stains positive for CD31 (10×) and (D) Fli1 (10×).