Background: Women with Lynch syndrome have a 40 to 60% lifetime risk of endometrial cancer and a 10 to 12% lifetime risk of ovarian cancer and may consider prophylactic gynecological surgery as an option for risk reduction.
Case: We report a case of synchronous primary cancers of the endometrium and fallopian tube diagnosed at time of prophylactic surgery in an MSH2 mutation carrier.
Conclusion: Risk-reducing gynecological surgery in Lynch syndrome must include complete removal of the fallopian tubes in addition to the ovaries and endometrium, followed by careful pathological review. Prospective studies are needed to clarify the incidence of occult primary carcinoma of the fallopian tube among female MMR mutation carriers undergoing prophylactic surgery.