Chemotherapy and targeted treatments of breast sarcoma by histologic subtype.

: Breast sarcomas (BS) are uncommon and often present both diagnostic and therapeutic challenges. Historically, radical surgery has been the mainstay of treatment for localized breast sarcomas. On the other hand, in advanced disease, since they are a heterogeneous group of neoplasms consisting of several different subtypes including angiosarcoma, phyllodes tumor, and pleomorphic undifferentiated sarcoma, there is a lack of proven specific therapy. As a result, their treatment is based on the soft tissue sarcoma (STS) paradigm, whereas histotype-tailored approaches apply to specific subtypes like dermatofibrosarcoma protuberans. To date, advanced stages constitute an incurable form of disease and chemotherapy remains the cornerstone of treatment with the aim of palliation of symptoms and increase in survival.: In this manuscript, we review the clinicopathologic characteristics of the most common subtypes of BS, as well as the current treatment landscape of BS, with a particular focus on opportunities and challenges provided by new targeted molecules and immunotherapy.: The treatment approach of advanced BS is based on the pathologic subtype. A true breakthrough has still to be obtained, as the development of new agents in BS suffers from the same weaknesses as in other STS.