RESUMO
BACKGROUND: Breast Angiosarcoma is a rare type of malignancy arising from endothelial cells lining blood vessels, accounting for 1% of all soft tissue breast tumors. This retrospective study describes the clinical pathological features and clinical management and outcomes of a series of 5 patients with primary and secondary Angiosarcoma of the breast present to King Faisal Specialty Hospital and Research Center during the last 16 years. METHODS: A retrospective review of our institution's pathology database was conducted and all patients who had a pathologically confirmed breast angiosarcoma were included in this study. The patient's data, including demographic characteristics, pathological features, clinical management history and clinical outcomes were collected. RESULTS: Five patients were diagnosed with Breast Angiosarcoma (one secondary and four primary cases). The median age of patients with primary angiosarcoma was 22 years (range 13-25 years). All primary cases were presented late as post-excisional biopsy at local hospitals. Median tumor size was 6cm (range 4.0-17.0cm). All primary angiosarcoma patients had total mastectomy. Three-year disease-free survival (DFS) of patients with primary angiosarcoma was 25%. 5-year surviving rate of primary angiosarcoma was 50%. Recurrence was observed in three of the patients with primary Angiosarcoma and in the case of post irradiation Angiosarcoma. CONCLUSIONS: Our study demonstrates that Breast Angiosarcoma exhibits high recurrence and mortality rates. Early detection, small tumor size, and clear surgical margins seem to be crucial factors for survival. Mastectomy with adequate tumor margin is recommended and close long-term follow-up is of utmost importance. Surgery for local recurrence may be potentially curative.
