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• Pre-operative evaluation can decrease risk of operating on an unsuspected malignancy; morcellation of a sarcoma should be avoided. • Adjuvant treatment of early stage leiomyosarcomas is controversial with no convincing evidence that adjuvant chemotherapy improves outcomes outcomes. • Multiple chemotherapy agents, radiotherapy, and hormonal agents have activity in recurrent leiomyosarcoma. [ABSTRACT FROM AUTHOR]

Uterine carcinosarcoma (UCS) is a rare but aggressive cancer. In early-stage disease data guiding treatment is sparse. The purpose of this review is to summarize the findings from the 2019 NRG oncology group summer symposium meeting as well as a review of the current literature, with a particular focus on molecular targets, ongoing clinical trials, and treatment of early and advanced/recurrent disease. A combination of expert presentations and an extensive literature search was undertaken to summarize the literature in this review. MEDLINE was queried for peer-reviewed publications on UCS. This search was not limited by year or study design, but was limited to English language publications. ClinicalTrials.gov was queried for ongoing trials in UCS. UCS is a rare cancer that is biphasic, with the carcinomatous component driving its aggressive nature. Level 3 evidence regarding early stage disease is lacking, but retrospective data suggests adjuvant therapy is warranted. The recent results of GOG 261 have contributed valuable information towards treatment strategy, including use of paclitaxel and carboplatin for UCS. Clinical trials are ongoing to investigate new targeted agents in UCS. Ongoing endometrial cancer clinical trials now include UCS patients. In combination with advances in molecular profiling, this will provide patients with UCS improved therapeutic options. Until that time, surgical resection and traditional cytotoxic chemotherapy remains standard of care. • Although UCS is a rare, biphasic tumor, the carcinomatous component drives its aggressiveness. • Early stage UCS requires adjuvant chemotherapy, radiation, or a combination of both after surgical cytoreduction. • Ongoing clinical trials are evaluating a variety of molecular targeted drugs in UCS. [ABSTRACT FROM AUTHOR]