Multilocular Pure Leydig Cell Tumor of Ovary, Fallopian Tube, and Extraovarian Soft Tissue

ABSTRACT Objective: Leydig cell tumors (LCTs) of the ovary may produce androgens and cause virilization. Although they are generally benign, these tumors are typically very small, making them hard to detect by imaging processes. Methods: We report a case of a multilocular LCT involving the ovarian stroma, fallopian tube, and extra-ovarian soft tissue. It was diagnosed by catheter blood sampling of ovarian and adrenal venous blood. Results: A 63-year-old female presented to the endocrinology department with progressive hirsutism and male pattern alopecia occurring within 1 year. Laboratory tests revealed high serum testosterone. Diagnosis of an androgen-producing tumor was considered, however computed tomography and magnetic resonance imaging scans did not show any conspicuous results. Gynecological examination showed slightly enlarged ovaries. Ovarian and adrenal venous blood sampling was performed via catheter for further diagnostics. The testosterone concentration from the right ovarian vein was highly elevated. The patient was admitted for surgery to the gynecological department and bilateral adnexectomy was performed. Microscopic examination showed a multilocular LCT of the right ovary which was located in the ovarian stroma, the fallopian tube, and the extraovarian soft tissue. Following the surgery, her hirsutism disappeared and serum testosterone decreased to normal levels. Conclusion: LCTs typically present with postmenopausal virilization. Catheter blood sampling is a reliable method for diagnosis. Furthermore, follow up is essential as ovarian LCTs often have multilocular presentation. Abbreviations: GnRH = gonadotropin-releasing hormone; LCT = Leydig cell tumor