Mineralocorticoid Excess Secondary to Adrenal Cortical Carcinoma

Abstract

Adrenal cortical carcinoma may present solely with a syndrome of mineralocorticoid excess. Primary aldosteronism, resulting from adrenal carcinoma, is unusual and has only been reported rarely. A review of the literature revealed 19 cases with marked hypertension and hypokalemia, resulting from mineralocorticoid excess produced by adrenal cortical carcinoma. We report an additional case that supports the hypothesis that adrenal carcinoma may mimic hyperaldosteronism. Extensive assay of adrenal function will demonstrate the presence of biologically inactive steroids, suggesting the presence of adrenal cortical carcinoma.

Co-Secretion of Aldosterone and Cortisol by an Adrenocortical Carcinoma

“…The most common clinical syndrome associated with a functional adrenocortical carcinoma was Cushing’s syndrome, as reported in literature [1, 2, 3]. However, aldosterone-producing adrenocortical carcinoma is rare [4, 5, 8, 9]. Our case presented with hyperaldosteronism.…”

“…Although aldosterone may be the only adrenal steroid overproduced early in the course of the disease, hypersecretion of cortisol usually occurs at some point [1, 3]. It may also cause hyperandrogenism [3, 5]. Some patients only demonstrate excess precursor steroids in the absence of endocrine syndromes.…”

Aldosterone-producing adrenocortical carcinoma: an unusual cause of Conn’s syndrome with an ominous clinical course

“…The second, despite a smaller size and no histopathological features of malignancy, which suggested an initial diagnosis of Conn's adenoma, exhibited an ominous clinical course that led to the patient's death in 26 months. Sixty further cases of APAC could be identified in the literature (Foye 1955, Conn et al 1964, Crane et al 1965, Santander et al 1965, Alterman et al 1969, Brooks et al 1972, Filipecki et al 1972, Six et al 1972, Miyazaki et al 1973, Salassa et al 1975, Revach et al 1977, Boers et al 1981, Grim et al 1981, Taylor et al 1982, Slee et al 1983, Telner 1983, Arteaga et al 1984, Greathouse et al 1984, Levine et al 1984, Luscher et al 1984, Sakashita et al 1984, Stone et al 1984, Scott et al 1986, Valentini et al 1986, Farge et al 1987, Isles et al 1987, Tenschert et al 1987, Weiss et al 1989, Touitou et al 1992, Barksdale et al 1993, Boscaro et al 1995, Weingartner et al 1995, Sakai et al 1997, Taylor et al 1997, Muthusethupathi et al 1998, Siren et al 1998, Yamazaki et al 1998, Deckers et al 1999…”

Aldosterone-Producing Adrenal Cortical Cancer: A Case Report and Analysis of Steroidogenic Enzymes in the Tumor.

“…Biochemical data which suggest excess secretion of glucocorticoid such as increased excretion of UFC and the level of plasma cortisol not suppressed by DXM, were also observed in the present case. The concurrent secretion of other steroids may indicate the malignant potential of the tumor if this finding was observed in cases with hyperaldosteronism [11,12]. Plasma levels of other mineralocorticoids were not increased in the present case, but there are several reports of a concurrent increase in plasma corticosterone and DOC levels [11,12].…”

“…The concurrent secretion of other steroids may indicate the malignant potential of the tumor if this finding was observed in cases with hyperaldosteronism [11,12]. Plasma levels of other mineralocorticoids were not increased in the present case, but there are several reports of a concurrent increase in plasma corticosterone and DOC levels [11,12]. Plasma and/or urinary steroid levels may correlate with the activity of steroidogenic enzymes and the bulk of the tumor, and from this point of view, the biochemical measurement of enzyme activities in the tumor may play a role in investigating the character of steroidogenesis.…”