Primary Mammary Angiosarcoma: Literature Review

Angiosarcomas of the breast are extremely rare, highly aggressive tumors of vascular origin comprising 0.04% of all malignant neoplasms of the breast. They can be classified into primary mammary angiosarcomas and cutaneous (secondary) angiosarcomas. Primary angiosarcomas, owing to their unusual clinical presentation, are diagnosed late. In addition, the available literature to date lacks sufficient evidence to establish standard treatment guidelines for this group of tumors, thereby resulting in poor prognosis. In medical database, most available papers concern secondary angiosarcomas, with only a few case reports of primary angiosarcomas. The aim of this paper is to review what is known hitherto about the presentation, diagnostic tools, and therapeutic modalities for primary mammary angiosarcomas.