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LS\u2010associated endometrial cancer (LS\u2010EC) is the most common extraintestinal sentinel cancer caused by germline PVs in MMR genes, including <jats:italic>MLH1<\/jats:italic>, <jats:italic>MSH2<\/jats:italic>, <jats:italic>MSH6<\/jats:italic> and <jats:italic>PMS2<\/jats:italic>. The clinicopathologic features of LS\u2010EC include early age of onset, lower body mass index (BMI), endometrioid carcinoma and lower uterine segment involvement. There has been significant progress in screening, diagnosis, surveillance, prevention and treatment of LS\u2010EC. Many studies support universal screening for LS among patients with EC. Screening mainly involves a combination of traditional clinical criteria and molecular techniques, including MMR\u2010immunohistochemistry (MMR\u2010IHC), microsatellite instability (MSI) testing, <jats:italic>MLH1<\/jats:italic> promoter methylation testing and gene sequencing. 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