SUN-118 - Who Is the Culprit? A Rare Ovarian Cause of Extreme Hyperandrogenism

Background: Ovarian Sertoli-Leydig cell tumors (SLCT) are a rare sex cord–stromal tumors, accounting for < 0.2 % of all ovarian malignancies (1). They are more common in young women around 23 years of age or old but can be found in women of all age (2)(3). The age of the patient and stage of the disease are the most important factors in considering therapeutic management. As these tumors are found at an early stage in young women, fertility-sparing surgery is usually recommended (4). Here, we present an unusual case of hirsutism in a 30-years old patient.

Case presentation: The patient presented to the endocrine clinic for evaluation of amenorrhea and hirsutism of one year duration. She also reported virilization features including deepened voice, facial acne, increased muscle strength, clitoromegaly, hair thinning, hirsutism in upper lip, around the nipples and lower abdomen needing to shave every other day. Physical examination revealed a Ferriman-Gallwey score of 15. Laboratory results showed negative for B-HCG, testosterone levels of 384 ng/dl, Free testosterone of 54.5 ng/dl, however, Sex hormone binding globulin and DHEA levels were normal. FSH, LH, estradiol, prolactin, TSH, HbA1c, cortisol, androstenedione, were normal. Pelvic ultrasound revealed enlarged right ovary without any distinct mass. CT pelvis revealed right posterior adnexal mass measuring 3.9 cm x 3.4 cm with central necrosis concerning for neoplasm. MRI pelvis revealed 4.3 x 4.0 cm solid right ovarian mass with a small amount of degeneration, primary differential being an atypical stromal cell neoplasm with no findings of metastatic disease in pelvis. She successfully underwent right salpingo-oophorectomy. Histopathology revealed tumour sections mostly of Sertoli cells forming cords with focal open lumina and occasional Leydig cells with mild cytologic atypia.

Discussion: Sertoli-Leydig cell tumor is the most common virilizing ovarian tumor, with the usual and distinctive presenting complaints being abdominal pain and hormone-related symptoms. The prognosis of patients with grade 1 SLCT is excellent without adjuvant chemotherapy. Intermediate and poorly differentiated SLCTs require a more aggressive management. Our patient reported significant improvement in the symptoms after the surgery. This case is unique due to the rapid onset and severity of virilizing features in a premenopausal woman, highlighting the importance of having a broad differential diagnosis, including androgen-secreting tumors.

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