Endocrine Abstracts

Introduction: Ovarian hyperthecosis (OH) is a rare condition, reported only in case reports and small case series and is characterized by severe hyperandrogenism leading to virilisation and insulin resistance. The term hyperthecosis refers to the presence of luteinized thecal cells within a hyperplastic ovarian stroma and the pathophysiology of this remains poorly understood. Despite the fact that hyperandrogenism is a relatively common clinical problem, severe hyperandrogenism causing virilisation is rare.

Case report: a 40 year-old Caucasian woman with a prior history of PCOS presented to our department with clinical signs of hyperandrogenism (hirsutism – Ferriman-Gallwey score=28, androgenic alopecia with temporal and anterior baldness, clitoromegaly and deepening of the voice). Menarche occurred at 14 years old with irregular menses and she reported secondary amenorrhea at the age of 30. She was known to have type 2 diabetes and hypertension. Clinical examination revealed acanthosis nigricans on armpits and groin, purple stretch marks on breasts and she was obese with body mass index of 34 kg/m² with moon face and buffalo hump. Hormonal profile demonstrated elevated total testosterone 4.11 nmol/l (0.29-1.67), with dehydroepiandrosterone sulfate 134.5 (60-337), 17-hydroxyprogesterone 1.82 ng/ml, low follicular stimulating hormone (FSH) 8.03 mIU/ml and luteinizing hormone (LH) 6.79 mIU/ml and a serum estradiol in the premenopausal range 53.9 pgI/ml. The results of further diagnostic test were as follows: elevated urinary free cortisol 604.8 mg/24 h (9.5-148) but with normal cortisol in overnight dexamethasone suppression test (1.32 mg/dl). Thyroid function tests and prolactin were normal. Abdominal and pelvic CT scan indicated bilateral ovarian enlargement (right ovary: 36/60/55 mm and left ovary: 38/41/57 mm) with hypodense structure on unenhanced imaging showing mild contrast uptake. She underwent laparoscopic surgery with total hysterectomy and bilateral salpingooophorectomy. Anatomopathological examination confirmed diagnosis of OH describing ovarian cortex bilaterally expanded, with multiple follicular cysts in the superficial layer and scattered luteinized cells isolated or organized in small clusters.

Discussions: Even if is a considerable overlap between hyperthecosis and PCOS making difficult to distinguish between these two conditions, testosterone levels are higher than the levels observed in PCOS and since this modified parameter is the single most important laboratory finding, imaging of the adrenal glands and ovaries must be performed. A complete medical history and full physical examination for virilisation are important hallmarks of diagnosis. Effective and timely treatment can reverse the cardio-metabolic consequences of hyperandrogenemia as well as clinical and biochemical outcomes.