Primary high-grade ovarian sertoli–leydig cell tumor withbilateral adnexal involvement in a patient diagnosed withpeutz–jeghers syndrome

Abstract

AimPeutz-Jeghers syndrome is a rare genetic disease with an increased risk of gastrointestinal and extragastrointestinal malignancies. Ovarian involvement of Sertoli-Leydig cell tumors is uncommon and even more rare in Peutz-Jeghers syndrome patients. This case report outlines the importance of primary ovarian Sertoli-Leydig cell tumor with bilateral adnexal involvement in a patient diagnosed with Peutz-Jeghers syndrome.CaseA 31-year-old female patient diagnosed with Peutz-Jeghers syndrome presented to our clinic with pelvic pain. Ultrasound examination revealed solid masses in both adnexa. Laparoscopic fertility-preserving surgery was performed. Pathology confirmed the diagnosis of poorly differentiated solid ovarian Sertoli-Leydig cell tumor. Staging surgery was performed, and finally, the patient was referred to oncology for chemotherapy.ConclusionThis case report outlines the importance of Sertoli-Leydig cell tumors in the differential diagnosis of adnexal masses in patients with Peutz-Jeghers syndrome. Bilateral adnexal involvement is an unusual presentation of Sertoli-Leydig cell tumors. Despite unusual and different presentations, Sertoli-Leydig cell tumors should not be ignored or overlooked in patients with Peutz-Jeghers syndrome.