Carcinosarcoma of the breast: Facing the challenge of a rare nosologic entity

Carcinosarcoma (CS), also known as metaplastic breast carcinoma with mesenchymal differentiation, is one of the five distinct subtypes of metaplastic breast cancer. It is considered as a mixed, biphasic neoplasm consisting of a carcinomatous component combined with a malignant nonepithelial element of mesenchymal origin without an intermediate transition zone. Although cellular origin of this neoplasm remains controversial, most researchers declare that neoplastic cells derive from a cellular structure with potential biphasic differentiation. Despite recent research on the therapeutic strategies against CS neoplastic disorders, surgical resection appears the only potentially curative approach. Since CS metastasize by the lymphatic route, axillary assessment with sentinel lymph node biopsy and/or axillary lymph node dissection is always implemented. Nevertheless, the tumor also presents a hematogenous metastatic pattern including pleural, pulmonary, liver, brain and less commonly bone metastases. Thus, surgical removal of breast CS does not necessarily ensure patient's long-term recovery. Moreover, alternative therapies, such as radio- and chemotherapy proved insufficient and 5-year survival rate is limited. Nevertheless, there is evidence that following surgery, the combination of radio and chemotherapy is associated with a better prognosis than either treatment alone. The aim of this review is to evaluate the results of surgical treatment for breast CS with special reference to the extent of its histological spread. Clinical features, histogenesis, morphological and immunochemical findings are discussed, while the role of current diagnostic and therapeutic management of this aggressive neoplasm is emphasized.