Endocrinology Fellow Walter Reed National Military Medical Center Bethesda, Maryland, United States
Introduction: Ovarian steroid cell tumors (SCTs) are a rare type of sex-cord stromal tumors that account for 0.1% of all ovarian tumors. These tumors can occur at any age and typically secrete hormones such as androstenedione and testosterone. We report a young woman with known microprolactinoma who presented with secondary amenorrhea and virilization and was found to have a steroid secreting ovarian SCT.
Case Description: A 32-year-old nulligravid woman with history of infertility presented for evaluation for amenorrhea. Three years ago, she developed oligomenorrhea and was subsequently found to have hyperprolactinemia and an 8mm pituitary microadenoma. Thyroid function tests and IGF-1 at the time were normal, no other workup was obtained. She was started on cabergoline, though after developing full body hives was switched to bromocriptine. Despite prolactin normalization from 69.9 to 14.7 ng/mL and shrinkage of pituitary adenoma to 7mm after 1 year of therapy, she developed amenorrhea, acne, and new facial hair. Her testosterone was found elevated 212 ng/dL and she states was then diagnosed with PCOS. In light of no clinical improvement and mental health issues, she self-discontinued her bromocriptine and was lost to follow up. Upon current presentation she reported last menstrual period 24 months ago, continued acne and facial hair, and now temporal hair loss and deepening voice. Ferriman-Gallwey score was 20/36. She did not have clinical signs of Cushing’s syndrome. Repeat prolactin was 195 ng/mL, pituitary MRI showed 3mm microadenoma, and bromocriptine was resumed. Workup for her hyperandrogenism revealed testosterone of 137 ng/dL (ref 8-60), androstenedione 972 ng/dL (41-262), DHEA-S 284 mcg/dL (84.8-378), 17-OH-progesterone 23 ng/dL, and hCG <0.5mIU/mL. Late night salivary cortisol levels were elevated to 0.213, 0.429, and 0.552 mcg/dL ( <0.090) with ACTH 27.3 pg/mL (9.72-63.3). CT scan showed normal adrenal glands. A pelvic ultrasound revealed a 2.2 cm left ovarian vascular mass. She underwent left salpingo-oophorectomy and pathology confirmed a 2.4 cm benign steroid cell tumor. Follow up after surgery is currently ongoing.
Discussion: Amenorrhea despite prolactin normalization and signs of severe hyperandrogenism such as testosterone >150ng/dL, male pattern baldness, and vocal deepening should prompt physicians to consider androgen-secreting tumors. Our patient was found to have a rare ovarian SCT—these tumors can lead to precocious puberty in adolescents, virilization in adults, Cushing’s syndrome, and infertility. Our patient reports significant clinical improvement postoperatively.