Endocrine Abstracts

A 67-year-old lady was referred to Haematology for evaluation of Polycythemia (Haemoglobin 162 – 172 g/l, PCV 50 – 53%) A simultaneous referral was sent to Endocrinology for hirsutism, noting that her Testosterone level was 24 nmol/l, SHBG 33 nmol/l. Her past medical history was significant for hypothyroidism, hypertension and migraines for which she was on Levothyroxine, amlodipine and candesartan. She was a non-smoker and teetotaller. Her haematology workup was negative including a JAK2 mutation. It was thus understood that her polycythemia was secondary to hyperandrogenism. Hirsutism: Over the past 2 years, she noted increased facial hair, and increased hair on her shoulders, breasts, and abdomen (Ferriman-Gallwey score 9). Her BMI was 36 with central obesity but no features of Cushing’s syndrome; there was no cliteromegaly and she did not report any voice change. She had been menopausal since 20 years. Her hormonal profile showed LH 9.5 miu/l, FSH 31.6 miu/l, oestradiol 166 pmol/l. Prolactin, IGF1 and cortisol level were normal. US pelvis was unable to identify either ovary. A preliminary diagnosis of ovarian hyperthecosis was entertained, though given the high level of testosterone further imaging was requested to exclude ovarian or adrenal tumour. CT abdomen identified 1.6 cm right adrenal nodule (washout curves - indeterminate results) No nodule was seen in left adrenal gland. Both ovaries were visualised and were bulky for a post-menopausal woman, measuring 21 mm (right) and 26 mm (left). She was trialled on prostap 3.75 mg IM monthly. This decreased her Testosterone to 3.2 nmol/l, eventually leading to normal testosterone of 1.2 nmol/l. Alongside, a workup for adrenal incidentaloma showed suppressed cortisol on ONDST, normal aldosterone-renin ratio but marginally elevated plasma normetadrenaline 1357 pmol/l (normal 120 – 1180) confirmed on 2 samples. MIBG showed increased uptake in right adrenal gland in keeping with Pheochromocytoma. She underwent right adrenalectomy and the histology showed pheochromocytoma with low mitotic activity. Post-op, her plasma metadrenaline normalised completely and she only required amlodipine to manage her BP. Genetic testing for pheochromocytoma was negative. She stopped taking prostap after a couple of years as she felt it was related to weight gain. Though her Testosterone started to rise again, it was not to the same extent as before (Testosterone 5.2 – 8 nmol/l) with no recurrence of hirsutism. She was offered Prostap once polycythemia recurred. Long term management for ovarian hyperthecosis would be oophorectomy which she may consider.