Primary angiosarcoma of the breast

Primary angiosarcoma (AS) of the breast is a rare neoplasia that is not related to radiation exposure. It represents less than 0.05% of all malignant breast tumors. This lesion is characterized by aggressive patterns and poor prognosis and by the absence of typical features at radiologic examination. Currently there are not evidence-based guidelines regarding surgical and adjuvant treatment for this tumor even though wide surgical resection followed by chemo- radiotherapy appears to improve both disease free survival and overall survival. The aim of this study was to analyze the available series of AS patients suggesting the most reliable treatment options for this rare neoplasia.