Primary High-Grade Ovarian Sertoli-Leydig Cell Tumor With Bilateral Adnexal Involvement in a Patient Diagnosed With Peutz-Jeghers Syndrome

This case report discusses a 31-year-old patient with Peutz–Jeghers syndrome (PJS) who developed a rare primary high-grade ovarian Sertoli–Leydig cell tumor (SLCT) with bilateral adnexal involvement. PJS, a genetic condition, increases the risk of various cancers, including rare ovarian neoplasms like SLCTs, which are less than 0.5% of all ovarian tumors but rise to 20% in PJS patients. The patient presented with pelvic pain and was found to have atypical bilateral adnexal masses. Surgical intervention preserved fertility while removing the tumors, and subsequent chemotherapy showed no pathological findings. This case highlights the importance of considering SLCTs in PJS patients and suggests genetic screening for relatives to detect potential ovarian tumors early.