Context.-Endometrial carcinoma is a disease of older postmenopausal women, and is relatively uncommon in patients younger than 40 years. Endometrial carcinomas in this age group may be familial, associated with Lynch syndrome, or sporadic.Objectives.-To present our current knowledge of endometrial carcinomas in women younger than 40 years.Data Sources.-The review is based on previously published articles on this topic.Conclusions.-Most endometrial carcinomas that occur in this age group are associated with estrogen excess. They are usually low-grade endometrioid carcinomas that present at low stages and are associated with favorable clinical outcomes. Tumors associated with mismatch repair abnormalities and Lynch syndrome appear to be distinct, with worse prognostic factors and, possibly, clinical behavior. Conservative hormonal therapy and ovarian conservation are reasonable considerations in the management of these young patients, but carry the risk of tumor progression, recurrence, and an occult synchronous or metachronous ovarian carcinoma.
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