Aldosterone-Producing Adrenocortical Carcinoma

Abstract

In 1955 Conn described the syndrome of primary hyperaldosteronism (PHA) [1], which has come to be known as “Conn’s Syndrome” [2]. PHA is a group of disorders in which aldosterone production is inappropriately high, relatively autonomous from the renin-angiotensin system, and nonsuppressible by sodium loading. Such inappropriate production of aldosterone causes cardiovascular damage, suppression of plasma renin, hypertension, sodium retention, and potassium excretion that if prolonged and severe may lead to hypokalemia. PHA is commonly caused by adenoma (35%), bilateral adrenal hyperplasia (60%), or (rarely) by aldosterone producing adrenocortical carcinoma (APAC) (<1%) (Table 27.1) [3]. The first APAC was reported in 1955 by Foye and Feichtmeir [4] shortly after Conn’s original report. Aldosterone hypersecretion in adrenocortical carcinoma (ACC) is rare with only 58 patients being reported in a recent review [5]. The reported numbers of APAC amongst ACCs vary significantly. In one large series of ACCs, only 2.5% had developed hyperaldosteronism [6]. In a single center analysis on ACCs, which were subjected to operative management at the Mayo Clnic, the portion of APACs was 11%. Conversely, it has been estimated that hyperaldosteronism is due to APAC in only 1% of patients with PHA [7].