The Sertoli cell tumor of the ovary is a rare ovarian tumor with non-specific symptoms. According to the literature, endocrine manifestations occur in two-thirds of patients, but testosterone production is extremely rare. Typically, it is a unilateral benign tumor of the ovary that most commonly presents in adolescents and young women of childbearing potential. We report a 29-year-old patient, previously diagnosed to have polycystic ovarian syndrome, who presented with complaints of amenorrhea for the past three years. A transvaginal ultrasound scan revealed polycystic structure ovaries and a solid cystic formation of 32 × 31 mm size with strong blood flow in the left ovary. The laboratory tests reported an elevated testosterone level. During laparoscopic surgery, a solid, yellowish tumor was removed and the left ovary was resected. Histological examination revealed a left ovary Sertoli cell tumor with an immature prepubertal-like Sertoli cell component. Following surgery, the serum testosterone levels returned to normal and the menstrual cycle became regular. Due to the substantially low incidence of ovarian Sertoli cell tumors, information on their clinical behavior, morphologic spectrum, optimal management, and prognosis is limited. They are characterized by a wide variety of clinical manifestations, treated surgically, and, if diagnosed at an early stage, have good prognosis. We emphasize the extraordinarily rare clinical presentation of this case report.
Keywords: non-epithelial ovarian cancer; pure Sertoli cell tumor; sex chord tumor.