Adrenocortical carcinoma producing 11-deoxycorticosterone: A rare cause of mineralocorticoid hypertension

Abstract

A 37-yr-old man presented with the classic signs of mineralocorticoid excess hypertension and hypokalemia. The cause was not aldosterone excess, but elevation of plasma 11-deoxycorticosterone (DOC). Computed tomography (CT) scans showed a large right adrenal mass without signs of metastatic disease. The tumor was removed by open laparotomy, and histology revealed an adrenocortical carcinoma. Two yr after diagnosis, the patient is in good general condition and there is no sign of recurrence or metastatic disease, despite the large tumor size. DOC producing adrenocortical carcinomas causing mineralocorticoid hypertension are very rare, so far only 10 cases have been described in the literature.

Adrenocortical carcinoma

“…An uncommon diagnosis of mineralocorticoid excess has been reported recently by Mussig et al [9] in a man with Conn syndrome and isolated plasma 11-deoxycorticosterone (DOC) excess. Only 11 cases have been reported in the literature, making this isolated malignancy extremely rare.…”

“…Only 11 cases have been reported in the literature, making this isolated malignancy extremely rare. Combined mineralocorticoid and glucocorticoid secretion has also been reported in ACC, emphasizing the need for hormonal screening in patients with adrenal masses [9].…”

From β-Catenin to ARM-Repeat Proteins in Adrenocortical Disorders

“…Although mice with constitutive adrenocortical β-catenin activation develop aldosterone-producing tumors at 10 months of age, the plasma aldosterone concentration decreases with malignant progression, which in some cases is associated with an increase of plasma corticosterone concentration [44]. This is similar to rare human cases, in which patients with APA have later developed hypercortisolism [45][46][47]. Moreover, in cell culture, β-catenin knockdown induces a decrease of cortisol secretion in cell medium due to a preferential DHEA production [41].…”

Adrenocortical Carcinoma

“…Autonomous aldosterone secretion (which classically leads to hypertension and hypokalemia) is rare in ACC (85,86). More commonly, mineralocorticoid effects are mediated by high cortisol levels or possibly steroid precursors with mineralocorticoid activity, such as 11-deoxycorticosterone (87,88).…”