Abstract: Uterine leiomyosarcoma(ULMS) is an aggressive, rapidly progressive uterine malignancy lacking specific biomarkers of clinic screening, resulting in an extremely low preoperative diagnostic rate. The treatment of this malignant tumor is primarily surgery, supplemented by radiotherapy, chemotherapy, targeted therapy, hormonotherapy and biotherapy; however, there is no obvious curative effect. Recently, great advancement has been achieved in searching biomarkers related to the diagnosis and prognosis of ULMS from etiology. This review will investigate the etiology and mechanism of ULMS from the perspective of epidemiological studies, hormone, gene and molecular biomarkers, in order to provide new ideas for clinical diagnosis and treatment.