Abstract: Lynch syndrome (LS) is an autosomal dominant hereditary disease, formerly known as hereditary nonpolyposis colorectal cancer (HNPCC), which is caused by mismatch repair (MMR) gene mutations in the germline of MLH1, MSH2, MSH6 and PMS2. LS patients have a variety of canceration tendency, low age of onset and family susceptibility, and can develop colorectal cancer, endometrial cancer (EC), ovarian cancer, gastric cancer, and breast cancer at the same or different time. EC is most closely related to it in female patients. At present, there is still insufficient understanding of LS-EC in China, and no complete diagnosis and treatment standards or guidelines have been formed. Therefore, this paper reviews the molecular mechanism, clinicopathological characteristics, screening and diagnostic methods, clinical treatment methods and prevention of LS-EC to improve the understanding of LS-EC.

Key words: Lynch syndrome, Endometrial neoplasms, DNA mismatch repair, Genes；Diagnosis, Therapy