Uterine carcinosarcoma: A review of the literature

•UCS is an aggressive de-differentiated endometrial carcinoma with its own molecular profile.•Surgical staging and adjuvant chemotherapy are crucial for all stages of disease.•Future study should focus on UCS specifically and consider targeted therapies.

ObjectiveUterine carcinosarcomas (UCSs) are aggressive tumors previously considered to be sarcomas, but now recognized as malignancies composed of metaplastic transformation of epithelial elements. Much of the management for UCS has been extrapolated from studies of endometrial carcinomas and sarcomas. This article critically reviews the literature pertinent to the pathology, pathogenesis, diagnosis and management of women with UCS.MethodsMEDLINE was searched for English language literature on UCS with a focus on the past 20 years. Given the rarity of this tumor, studies were not limited by design or number of reported patients.ResultsUCS is biologically a de-differentiated endometrial carcinoma with its own pathogenesis and molecular profile. It commonly presents with extrauterine disease which can be identified by comprehensive surgical staging. Most UCS patients are candidates for adjuvant chemotherapy. The role of radiation is less clear. Combination therapy, while commonly used, has not been studied in depth. The high recurrence rate and poor overall survival for UCS suggest an ongoing need for clinical trials for UCS specifically.ConclusionsUCS represents a distinct subtype of uterine malignancy, and should be studied as such via focused clinical trials.