Diagnosis and management of a recurrent polymerase-epsilon (POLE)-mutated endometrial cancer

Polymerase-epsilon (POLE)-mutated carcinomas are a rare, but well-known subtype of endometrial cancer. While typically associated with good prognosis, recurrences are documented. Here we present a case of recurrent POLE-mutated endometrial cancer, discuss pathologic features, current methods of molecular classification, and explore therapeutic implications for the POLE-mutation phenotype. •POLE mutations occur in 7–12% of endometrial cancers and are identified by molecular analysis.•POLE-mutated endometrial cancers have high tumor mutation burden, tumor neoantigen production, and tumor infiltrating T cells.•This case illustrates a marked response to immune checkpoint inhibition in a POLE-mutated endometrial cancer.