Primary and secondary angiosarcoma of the breast: The Mayo Clinic experience

Background and Objectives: Angiosarcoma of the breast can be divided into primary and secondary. The objective was to determine clinicopathologic factors associated with breast angiosarcoma and to compare primary versus secondary angiosarcoma. Methods: Breast angiosarcoma cases at Mayo Clinic from 1960 to 2008 were identified. Clinicopathologic factors were reviewed. Characteristics of primary and secondary angiosarcoma were compared. Results: Twenty-seven cases of primary angiosarcoma and 14 cases of secondary angiosarcoma were identified. The median age of primary angiosarcoma patients was lower than that of secondary angiosarcoma - 43 years versus 73 years (P<0.0001). Primary angiosarcoma more frequently presented with a mass, whereas secondary angiosarcoma presented with a rash (P<0.0001). Median time from radiation to secondary angiosarcoma diagnosis was 6.8 years. Median tumor size was 7.0 cm for primary angiosarcoma and 5.0 cm for secondary angiosarcoma (P=0.7). Tumors were high grade in 33% of primary angiosarcoma and 82% of secondary angiosarcoma (P=0.02). Five-year survival for primary and secondary angiosarcoma was 46% and 69%, respectively (P=0.8). Conclusion: Primary angiosarcoma occurs in younger patients than secondary and more frequently presents with a mass. Mastectomy is the mainstay of treatment for breast angiosarcoma. Breast angiosarcoma is a rare malignancy with poor long-term prognosis.