Aldosterone-Producing Adrenocortical Carcinoma

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Abstract
Three patients with primary aldosteronism due to adrenocortical carcinoma were studied, 2 with hyperaldosteronism alone and 1 also with hypercortisolism; in the later stages all 3 had hypersecretion of glucocorticoid and androgenic hormones. Although clinical presentations were similar to those of patients with benign adenoma, all had significantly higher concentrations of deoxycorticosterone and aldosterone and more profound hypokalemia. Stimulation with ACTH in 2 patients showed a good cortisol response but no aldosterone response. The circadian rhythm for cortisol was normal but absent for aldosterone and deoxycorticosterone. Sequential 24-h circadian studies in 1 patient showed that as the disease progressed, corticosterone and finally cortisol lost their circadian rhythms. Treatment with spironolactone, mitotane or aminoglutethimide had transient clinical effects. The patients died 2-13 yr later.