A 62-year-old female presented to the emergency department with severe hypertension. Further questioning revealed a four-month history of low mood, poor concentration, lethargy and proximal weakness She also reported a 10kg weight gain and new facial hirsuitism over the preceding year.
On examination she was hypertensive (BP 215/99mmHg) on four antihypertensives with subtle cushingoid features. She demonstrated slight facial plethora, mild proximal myopathy, mild dorsocervical and supraclavicular fat-padding. There was no abdominal striae or appreciable abdominal masses. She had features of hyperandrogenism with Ferriman and Gallway scores of 3 and 2 at lip and chin respectively.
Biochemistry demonstrated marked hypokalaemia with potassium of 2.9mmol/L and an aldosterone level was 120pmol/L (<978), with a renin level of 15mIu/L (4.4 – 46) and a ratio of 8 (<70) (while taking interfering medications). Catecholamines and metanephrines were normal.
Total testosterone was markedly elevated to 7.0nmol/L (0.2 – 1.8), with DHEA-S and androstenedione levels on the upper limit of normal at 10umol/L (1.0 – 11.7) and 12.9nmol/L (1.0 – 12.9) respectively. Cortisol precursors were elevated with an 11-deoxycortisol level of 4.8nmol/L (0.2 – 4.6). Diurnal variation in cortisol secretion was lost with fasting levels from 1162-1281nmol/L and evening levels between 693 -1058nmol/L.
CT and MRI scans demonstrated a large bilobed right adrenal mass measuring 6.4 x 4.9 x 5.8cm with diffusion restriction increasing the likelihood of a malignant lesion/adrenal carcinoma.
She proceeded to an open adrenalectomy. Histopathology identified a 7.5cm adrenocortical carcinoma with fat and vascular invasion, a modified Weiss Criteria score of 6, and a Ki67 index of 15-20%.
Post operatively blood pressure improved to 130mmHg on two agents and potassium normalized off Spironolactone. Cortisol, testosterone and DHEAS levels declined precipitously in keeping with suppression of the contralateral adrenal gland. She was commenced on Mitotane therapy given the high-risk features of adrenocortical carcinoma.