Endocrine Abstracts

A 51 year old presented with headaches, fatigue and generalised weakness. She had a background history of hypertension diagnosed one year prior to presentation. Initial laboratory evaluation demonstrated life-threatening hypokalaemia, potassium 0.9mmol/l(R.I. 3.5-5.3). This profound hypokalaemia required ICU admission for replacement of potassium and monitoring. Management of subsequent fluid overload necessitated a brief period of hemofiltration. During the course of evaluation a 20 cm adrenal mass was discovered with nodal, hepatic and pulmonary lesions, which were FDG avid on PET imaging. Radiology was concerning for metastatic ACC and hormonal evaluation reported elevated 11-dexoycortisol (DOC) levels, 100 times higher than the upper limit of the reference. Overnight dexamethasone suppression test cortisol was 127nmol/lwith low adrenal androgens DHEAS 0.4 umol/l(1.0-7.0.), DHEA 2.1nmol/l(3.1 -33), Androstenedione 1.05 nmol/l(1.39-9.77) and low testosterone <0.4nmol/l. Aldosterone was 825 pmol/l(0-1179) with renin 8.5 mIU/l. Following multi-disciplinary input, open cytoreductive adrenalectomy and nephrectomy was planned. Pre-operatively hypokalaemia and hypertension were managed with oral potassium replacement, spironolactone, ramipril, amlodipine and doxazosin. Subsequent histology confirmed metastatic ACC with hepatic and nodal involvement, Weiss score 5/9, Ki67 score 20%. Her post-operative course has been complicated by decompensated heart failure aggravated by tumour related mineralocorticoid and glucocorticoid excess. Mitotane and replacement hydrocortisone have been initiated post-operatively with systemic chemotherapy planned in light of disease progression. First post-operative 11-DOC levels are suppressed. Persistent PTH-dependent hypercalcaemia has prompted genetic evaluation for MEN-1, results are awaited. This complex case highlights the complications of hormonal hypersecretion associated with ACC and the profound hypokalaemia that can result from 11-DOC hypersecretion.