A Case of Adrenocortical Carcinoma With Severe Hypertension and Hypokalemia

Abstract Case: 70 years old woman was pointed out for hypertension at a medical checkup and visited primary physician. Severe hypokalemia (2.4 mmol/L) noted, abdominal CT scan revealed right 99 mm diameter adrenal tumor adjacent to 80 liver tumor. She referred our hospital further management. Despite treatment with antihypertensive agents, including ARB, MRB, CCB, her blood pressure remained around 170/90 mmHg. In the meantime, 120 meq of potassium chloride needed keep serum levels 3.5 mmol/L. Both plasma renin activity (PRA) aldosterone concentration (PAC) were high (6.4 ng/mL/h 376 pg/mL), indicating renin-dependent hypertension. Since angiography renal artery compressed downward by tumor, renovascular suspected. Moreover, both early morning ACTH cortisol suppressed (8.5 pmol/L 5.3 μg/dL), despite elevated urinary free (350 μg/day). Urinary steroid profile analysis detected various precursor metabolites, deoxycorticosterone (DOC) corticosterone. DOC 5 folds more than upper normal limit, suggesting one causes hypokalemia, while corticosterone thought be contributed suppressing ACTH. After adrenalectomy hepatic segmentectomy, rapidly improved declining PRA PAC 0.2 < 0.7 pg/mL, respectively. also fell into range. From pathological findings, she diagnosed adrenocortical carcinoma (ACC) infiltrating (pT4N0M0, stage IV). The immunohistochemistry showed disorganized expression steroidogenic enzymes, CYP11B1, CYP11B2, 3β-HSD, supporting variety production metabolites from ACC. Although mitotane initiated as adjuvant chemotherapy, multiple metastasis found lung, liver, vertebrae, 6 months after operation. Along that, recurred gradually elevation. However, no apparent stenosis. We performed immunostaining ACC specimen partially positive, demonstrating this Conclusion: juxtaglomerular cell is most common renin-producing some cases secreting have been reported quite rare case. present case, secretion might severe stenosis induced are considered major cause.