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Xiaohong Yao,1 Min Feng,1,2 Wei Wang1,2 1Department of Pathology, West China Second University Hospital, Sichuan University, Sichuan, Chengdu, People’s Republic of China; 2Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, Sichuan, People’s Republic of ChinaCorrespondence: Wei Wang, Email bearwin2000@126.comAbstract: Endometrial cancer shows high histological and molecular heterogeneity. The POLE mutation is a significant molecular alteration in endometrial cancer, leading to the identification of a specific subtype known as POLE-mutated endometrial cancer. This subtype exhibits a high tumor mutation burden, abundant lymphocyte infiltration, and a favorable prognosis, making it a promising candidate for immune checkpoint inhibitor therapy. This paper presents a comprehensive review of the clinical and pathological characteristics, outcomes, treatment advancements, pathogenic POLE gene detection, and alternative testing methods for POLE-mutated endometrial cancer.Keywords: endometrial cancer, molecular subtyping, POLE, POLE-mutated endometrial cancer, immunotherapy