Benign Desoxycorticosterone-Producing Adrenal Tumor

Kondo, Kazuoki

doi:10.1001/jama.1976.03270100042026

Cited by 12 publications

(7 citation statements)

References 14 publications

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“…This finding is consistent with earlier reports indicating that plasma DOC levels are under the control of ACTH in benign DOC-producing tumors, but not in malignant tumors [3,4,[15][16][17]. Dexamethasone suppresses adrenal steroidogenesis by suppressing ACTH secretion from the pituitary gland.…”

Section: Discussionsupporting

confidence: 93%

“…Despite the patient's normal aldosterone in that case, corticosterone was hypersecreted, and hypertension and hypokalemia were observed. The first case of a DOC-producing adrenocortical carcinoma was reported in 1974 [3], and a benign DOCproducing adrenocortical adenoma was reported in 1976 [4]. Overall, however, the total number of the report of adrenal tumor producing mineralocorticoid intermediates was not so large even now.…”

Section: Introductionmentioning

confidence: 99%

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Close examination of steroidogenesis disorders in a DOC- and progesterone-producing adrenocortical carcinoma

Sone

Shibata

Homma

et al. 2008

Endocr

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We report a case of hypertension, hypokalemia, and amenorrhea accompanying an adrenocortical carcinoma. A 27-year-old woman was admitted to our hospital because of a left adrenal incidentaloma. She presented with hypertension, hypokalemia, and amenorrhea; her plasma renin activity was low, but her plasma aldosterone concentration was normal, as were cortisol and androgens. By contrast, her serum concentrations of deoxycorticosterone (DOC), 18-hydroxydeoxycorticosterone, and progesterone were high, and her urinary steroid profile showed elevated secretion of 17-deoxysteroids and 11-deoxysteroids (progesterone, DOC, 11-dehydrocorticosterone, and 11-deoxycortisol), and 3beta-hydroxy 5-en steroids (pregnenolone, 17-hydroxypregnenolone, and DHEA). Decreased ratios of metabolites of (1) 17-OHpregnenolone to pregnenolone and 17-OHprogesterone to progesterone, (2) corticosterone to DOC and cortisol to 11-deoxycortisol, and (3) progesterone to pregnenolone, 17-OHprogesterone to 17-OHpregnenolone and androstenedione to DHEA suggested the impairment of 17alpha-hydroxylase, 11beta-hydroxylase, and 3beta-HSD activities, respectively. After the tumor was removed, levels of all adrenal steroids were normalized. Based on the Weiss criteria, the tumor was diagnosed as an adrenocortical carcinoma, and immunohistochemical analysis of steroidogenic enzymes revealed disorganized steroidogenesis in the tumor tissue. With adrenocortical carcinomas, heterogeneity of individual steroid producing enzymes within tumor cells can lead to hypersecretion of various steroid intermediates, even when steroid end products are within the normal range.

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Section: Discussionsupporting

confidence: 93%

Section: Introductionmentioning

confidence: 99%

Close examination of steroidogenesis disorders in a DOC- and progesterone-producing adrenocortical carcinoma

Sone

Shibata

Homma

et al. 2008

Endocr

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“…97,[115][116][117] Other factors must also be considered to explain why in some cases patients do not develop hypertension but only edema and hypokalemia. [117][118][119][120][121][122][123][124][125][126][127][128][129] The latter is not always present and many subjects are normokalemic. After withdrawal from the substance, the usually symptoms disappear within 2 weeks; however, hypertension may remain without a clinical picture of pseudohyperaldosteronism.…”

Section: Licorice Derivatives (Glycyrrhizic Acid Glycyrrhetinic Amentioning

confidence: 99%

“…10,[128][129][130][131][132][133] The reduced or absent production of cortisol blocks the feedback regulation of ACTH, and, as a consequence, there is an increase in ACTH and in all the steroids that precede the blocked enzyme. 133 In these syndromes the high concentrations of free DOC allows the binding of the steroid to renal MR with subsequent volume expansion, suppression of renin and aldosterone, and hypertension.…”

Section: Table 3 Classification Of Desoxycorticosterone-related Causementioning

confidence: 99%

“…133 In these syndromes the high concentrations of free DOC allows the binding of the steroid to renal MR with subsequent volume expansion, suppression of renin and aldosterone, and hypertension. [127][128][129][130][131][132][133][134][135][136][137][138] In patients with deficiency of 11β-hydroxylase the conversions of 11OH-cortisol to cortisol and of DOC to corticosterone are blocked, while concentrations of DOC and of adrenal androgens are elevated. Two different 11β-hydroxylases have been described: CYP11B1 is regulated by ACTH, is located in the zona fasciculata, and converts 11OH-cortisol to cortisol, the second is located in the zona glomerulosa (CVYP11B2) and converts DOC to corticosterone.…”

Section: Table 3 Classification Of Desoxycorticosterone-related Causementioning

confidence: 99%

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Pseudohyperaldosteronism: Pathogenetic Mechanisms

Armanini

Calò

Semplicini

2003

Critical Reviews in Clinical Laboratory Sciences

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Pseudohyperaldosteronism is characterized by a clinical picture of hyperaldosteronism with suppression of plasma renin activity and aldosterone. Pseudohyperaldosteronism can be due to a direct mineralocorticoid effect, as with desoxycorticosterone, fluorohydrocortisone, fluoroprednisolone, estrogens, and the ingestion of high amounts of glycyrrhetinic acid. A block of 11-hydroxysteroid-dehydrogenase type 2 (11HSD2), the enzyme that converts cortisol into cortisone, at the level of epithelial target tissues of aldosterone, is involved in other cases. This mechanism is related either to a mutation of the gene, which encodes 11HSD2 (apparent mineralocorticoid excess syndrome and some cases of low renin hypertension) or to an acquired reduction of the activity of the enzyme due to glycyrrhetinic acid, carbenoxolone, and grapefruit juice. In other cases saturation of 11HSD2 may be involved as in severe Cushing's syndrome and chronic therapy with some corticosteroids. Recently, an activating mutation of the mineralocorticoid receptor gene has been described. Another genetic cause of pseudohyperaldosteronism is the syndrome of Liddle, which is due to a mutation of the gene encoding for beta and gamma subunits of the sodium channels.

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