Endocrine Abstracts

18 year old female referred with 2 years history of hirsutism and secondary amenorrhoea. She was born at 37 weeks and was found to have neonatal hypothermia, hypoglycaemia and crossed hemihypertrophy, but no macroglossia. Due to the increased risk of Wilm’s tumour she had regular ultrasound scans up to the age of 11 years. She attained menarche at the age of 13 but had only two periods within 18 months. By the age of 15 she developed hirsutism and acne. Biochemistry revealed serum Testosterone 5.3 nmol/l, SHGB 11 nmol/l, DHEA-sulphate >27 μmol/l, Androstendione >35 nmol/l. MRI scan showed bilateral adrenal masses measuring 8 and 6 cm in diameter. Staging CT scan confirmed no metastases. She underwent bilateral adrenalectomy. Post operatively she was commenced on Hydrocortisone and Fludrocortisone. She had her first menstrual cycle 2 months after surgery and serum androgens fell to undetectable levels. Histology of the adrenal masses showed adrenocortical neoplasm of uncertain malignant potential. The molecular basis of her condition is not yet known, and she did not have sufficient features to diagnose Beckwith-Weidemann syndrome, but adrenal tumours have been associated with overgrowth/hemihypertrophy syndromes, and continuing screening in adulthood should be considered.