PMON228 The Hidden Source of Testosterone Hypersecretion in a Female - a Thirty-Year Journey
Authors: Yang Almira , Firek Anthony , Munir Iqbal 
Published: November 2022

A Sertoli-Leydig cell tumor (SLCT) is a rare solid ovarian tumor that over-secretes testosterone or its precursor, androstenedione, that causes rapidly progressing severe androgen excess in females. A SLCT is usually large when patients seek medical assistance for hyperandrogenism. Testosterone production of a SLCT may be suppressed by GnRH agonists, making it unreliable to differentiate SLCT from another functional state. Ultrasound, CT, and MRI are usually used to visualize the ovarian tumor. We present a case of a female with severe hyperandrogenism from a SLCT taking three decades to confirm and resolve since conventional imaging could not detect the tumor.

A 58-year-old female was seen for unresolved hyperandrogenism. She first noted amenorrhea at the age of 30. Subsequently, she developed hirsutism with baldness, clitoromegaly, deepened voice, and masculinity over the next decade. The patient reported normal puberty and a history of regular menses. The patient achieved spontaneous pregnancies, which she carried to the term for the first two and had an ectopic pregnancy for the third. She reported a history of left salpingo-oophorectomy at 24, resulting from the ectopic pregnancy. Initial evaluation showed testosterone 370 ng/dL (15-70 ng/dL) with low LH. ACTH, cortisol, androstenedione, and 17-hydroxyprogesterone were normal. The initial pelvic ultrasound did not visualize ovarian or adnexal masses, nor did the abdominal and pelvic CT scan show any tumor. The patient underwent right salpingo-oophorectomy in 2005 to treat persistent hyperandrogenemia, which revealed normal pathology without resolution of her problem. A following transvaginal US showed an absence of ovaries bilaterally. She was treated with GnRH analog for an unclear period. Since establishing care in our endocrinology clinic two years ago due to changing insurance, her testosterone ranged 292-346 ng/dL and FSH and LH remained low. Her ACTH, cortisol, DHEA, DHEA-S, androstenedione, estradiol, and SHBG were normal. The repeat abdominal and pelvic CT scan was unremarkable. An abdominal MRI showed no adrenal lesions. The 48-hour low-dose dexamethasone suppression test suggested extra-adrenal hypertestosteronism. Finally, a PET-CT scan showed hypermetabolic activities in the left adnexal area. The patient eventually underwent removal of the left adnexal mass detected in the PET scan. The pathology showed a Leydig cell tumor. Postoperative total testosterone level dropped to 5 ng/dL.

Our patient presented with severe hyperandrogenism in her early 30s. Despite all biochemical evaluations suggesting SLTC, the conventional imaging studies failed to identify the tumor, and prior bilateral salpingo-oophorectomy failed to resolve her problem. Finally, PET/CT scan demonstrated an area of hypermetabolic activity in the left adnexal area, which was found to be a Leydig cell tumor after resection. While US, CT, and MRI remain the primary diagnostic tools for ovarian cancer, PET-CT should be considered when the lesion is not readily seen on conventional imaging studies.

Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.