Long-Standing Primary Aldosteronism in a Patient Diagnosed With Metastatic Adrenocortical Carcinoma

Most cases of primary aldosteronism (PA) are the results of either an aldosterone-producing adenoma or bilateral hyperplasia. Adrenocortical carcinoma (ACC) is a very rare cause of PA, accounting for <1% of all cases.

Case Report

A 49-year-old man was referred to our clinic for management of metastatic ACC. As highlighted in the text that follows, he had a remarkable history leading to current evaluation.

26 YEARS BEFORE CURRENT EVALUATION

He developed hypertension and had an evaluation for secondary causes of hypertension at our institution. At the time of initial evaluation, his blood pressure was 156/108 mmHg. His medications included metoprolol, hydrochlorothiazide, amlodipine, and Accupril. In addition, because of hypokalemia, he was taking 20 mEq of potassium chloride daily. Workup for PA was performed with a positive case detection and confirmatory testing ( Table 30.1 ). He was advised to proceed with abdominal imaging and consider adrenal venous sampling if interested in a potential surgical cure. At that time, he declined and elected therapy with spironolactone. He continued his care locally. Imaging was not performed.

TABLE 30.1

Laboratory Tests

	Age 23	Age 49
Biochemical Test	Result	Result	Reference Range
Cortisol, mcg/dL		19	7–21
ACTH, pg/mL		37	7.2–63
DHEA-S, mcg/dL		29	32–395
Pregnenolone, ng/dL		304	33–248
17-Hydroxypregnenolone, ng/dL		33	55–455
17-Hydroxyprogesterone, ng/dL		47	<220
11-Deoxycortisol, ng/dL		109	10–79
11-Deoxycorticosterone, ng/dL		447	<10
Total testosterone, ng/dL		388	240–950
Aldosterone, ng/dL	11	87	<21
Plasma renin activity, ng/mL per hour	<0.6	<0.6	≤0.6–3
24-Hour urine cortisol, mcg/24 h		62	<45
24-Hour urine aldosterone, mcg/24 h	19	264	<10
24-Hour urine sodium, mmol/24 h	267	375	41–227