What Should a Gynecologist Know about Li-Fraumeni Syndrome? Lessons from a Patient Undergoing Hysterectomy for Benign Indications.

Li-Fraumeni syndrome (LFS) is a rare highly penetrant cancer syndrome characterized by mutation in the TP53 tumor suppressor gene. Recent data suggest that this germline mutation is more frequent than once thought. While LFS has not been associated previously with pelvic serous carcinoma, gynecologic malignancies have been reported in this patient population. We present the case report of a 37-year-old patient with known LFS and a history of multiple cancers who underwent total abdominal hysterectomy for benign indications with incidental bilateral salpingo-oophorectomy. On final pathology, she was found to have serous tubal intraepithelial carcinoma of bilateral fallopian tubes. Our findings raise the question of the potential role of prophylactic gynecologic cancer-reducing surgery in this patient population.