Peutz–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.
A 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.
This 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.