Angiosarcoma of the breast following surgery and radiotherapy for breast cancer.

Breast angiosarcoma following surgery and radiotherapy for breast cancer is a rare but important clinical entity. This article reviews all published cases and includes data on incidence, etiology, presentation, diagnosis, management and prognosis. Breast angiosarcoma remains challenging clinically, radiologically and histologically, and thus a high index of suspicion is required in susceptible patients. Surgery is the primary treatment option and there are an increasing number of studies on the use of radiotherapy and chemotherapy, each with variable success. There have been recent reports of patients with metastatic disease responding to taxane chemotherapy, and there might be a future role for targeted agents given the expression of c-KIT in a subset of angiosarcomas. Overall survival, however, remains poor.