Endocrine Abstracts

Myxoid adrenocortical adenoma is a rare adenoma originated from adrenal cortex. A 51 years old male patient was admitted to our hospital due to hypertension and hypokalemia. He has a long history of nonregulated hypertension despite multiple antihypertensive medications. Hypokalemia was detected in last two years with potassium levels around 3.0 mmol/l on high supplementation dose up to 10–16 g of KCl/day. He is obese in the past 10 years. His family history was positive for diabetes (mother) and negative for hypertension. On addmition his blood pressure was 180/90 mmHg, pulse 92/min. Ambulatory blood pressure monitoring (ABPM) showed the mean blood pressure during the day of 159/88 mmHg, pulse 91/min, and during the night 158/85 mmHg, puls 79/min, without diurnal pattern. On 15 g of KCl/day, potassium level was 3.3 mmol/l, with 24 h urinary potassium 225 mmol/24 h. His urinary noradrenaline was above the upper limit (600.1 nmol/l, normal <570 nmol/24 h), while adrenaline (6.2 nmol/24 h) and dopamine (2017.3 nmol/24 h) were normal. Markers of neuroendocrine secretion CgA (23.9 ng/ml) and NSE (6.7 ng/ml) were normal. In LDDST cortisol was suppressed on 77 nmol/l. Testing of the RAAS showed high aldosterone as well as high plasma renin activity (aldosterone >1400 ng/l; PRA 12.1 ng/ml per h; K 4.0 nmol/l). Abdominal ultrasound, CT scan and MR describe hypervascular tumor in the right adrenal, 50×34×36 mm, ddg adrenal adenoma/pheochromocytoma. After premedication with phenoxybenzamine, patient was referred to surgeon who performed right adrenalectomy. Histopathological study showed several growth and distribution patterns. The dominant pattern was presented by cuboid, clear, eosinophilic cells with regular nucleus that were arranged in anastomosing and pseudoglandular patterns, within an abundant myxoid stroma. Immunohistochemical examination showed positivity of the tumour cells for CK AE1/AE3, CD56, inhibin and melanin A. Ki67 was 3%. EMA, CK20, CK7, calretinin and synaptophysin were negative. Weiss score:1. Dg: Pseudoglandular myxoid adrenal cortical adenoma. Postoperative testing showed normal urinary catecholamine levels, normal potassium and normal aldosterone levels with upper normal PRA (K 4.0 mmol/l; aldosterone 38.1 ng/l; PRA 6.49 ng/ml/h. ABPM on Ca-channel blocker, β-blocker and sartane showed normal diurnal pattern with the mean blood pressure during the day of 128/73 mmHg, pulse 69/min, and during the night 103/59 mmHg, puls 59/min. Up to present, 57 cases (24 cases of myxoid adrenocortical adenomas, 2 cases of borderline myxoid adrenocortical neoplasms and 31 cases of myxoid adrenocortical carcinomas) have been reported. Among them, 24 cases of these tumors were pseudoglandular pattern.