Angiosarcoma of the breast

A case of angiosarcoma of the breast with a fatal outcome is reported, and the world literature on this rare type of breast tumor is reviewed. Familiarity with the clinical and pathologic features of this entity is essential in avoiding underdiagnosis and delay of definitive treatment. The three-year survival rate of reported cases was 38%. However, the three-year, disease-free survival was only 14%. Five cases with five-year, disease-free survival have been reported to date. Eighteen patients (21%) showed contralateral breast involvement; in half of these, it occurred before other evidence of tumor dissemination. Simple mastectomy appears to be the optimal treatment of choice, since axillary node involvement is extremely rare. The role of irradiation and chemotherapy as adjuvant therapy remains to be evaluated.