Endocrine Abstracts

A 69-year-old woman was referred with a 2 year history of frontal balding, deepening voice and weight loss. Past medical history included primary hypothyroidism, osteoarthritis, varicose veins and menopause at age of 50 years. Current medications included levothyroxine. She was otherwise very well and enjoyed heightened physical endurance – regularly cycling >60 miles with a group of male friends. On examination she was virilised with male pattern alopecia, marked facial hirsutes, clitoromegaly and had a lean muscular build but no other abnormalities. Testosterone was elevated at 46.9 nmol/l (0.1–1.4). This was cross analysed for interference and confirmed with mass spectrometry. Dihydrotestosterone was 1.2 nmol/l (post-menopausal range <0.62); 17 hydroxyprogesterone was 16 nmol/l; SHBG 93 nmol/l (18–144); DHEAS 1.2 umol/l (0.7–11.5); androstenedione 8.7 nmol/l (1–11.5); FSH 17.7 IU/l (post-menopausal range 26.7–133.4), LH 4.6 IU/l (post-menopausal range 5.2–62), estradiol 256 pmol/l (post-menopausal range <183) indicating probable shunting of the androgens to oestrogen. There was no suppression of her androgens after standard low dose dexamethasone testing. MRI of the adrenals and pelvis revealed a heterogeneous 40 mm left ovarian mass, with normal adrenal appearance, and no evidence of distant disease. She underwent bilateral salpingo-oophorectomy. Histology and immunohistochemistry of the left ovarian lesion was consistent with a malignant steroid cell tumour with infiltration of the capsule but Ki 67 index of only 3%. There was positive staining for calretinin, inhibin and CD99. Following surgery her testosterone level is undetectable, her oestrodiol has also fallen and her physical appearance is gradually improving. Her case has been reviewed in a supraregional MDT and she is being closely monitored with serial serum testosterone and imaging. Ovarian steroid cell tumours are rare sex cord-stromal tumours with malignant potential. They account for <0.1% of all ovarian tumours. They are divided into three main subtypes including stromal luteoma, Leydig cell tumours, and steroid cell tumour NOS (not otherwise specified), which have uncertain cell lineage. Steroid cell NOS comprise more than 50% of steroid cell tumours, and may produce testosterone (most commonly), estradiol or cortisol. Up to 43% may be malignant and so long term monitoring is recommended. However, there are very limited numbers of cases reported and so long term prognosis and the duration of optimal follow up are not yet clear.