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Abstract
BackgroundCo-existing Cushing’s syndrome and primary aldosteronism caused by bilateral adrenocortical adenomas, secreting cortisol and aldosterone, respectively, have rarely been reported. Precise diagnosis and management of this disorder constitute a challenge to clinicians due to its atypical clinical manifestations and laboratory findings.Case presentationWe here report a Chinese male patient with co-existing Cushing’s syndrome and primary aldosteronism caused by bilateral adrenocortical adenomas, who complained of intermittent muscle weakness for over 3 years. Computed tomography scans revealed bilateral adrenal masses. Undetectable ACTH and unsuppressed cortisol levels by dexamethasone suggested ACTH-independent Cushing’s syndrome. Elevated aldosterone to renin ratio and unsuppressed plasma aldosterone concentration after saline infusion test suggested primary aldosteronism. Adrenal venous sampling adjusted by plasma epinephrine revealed hypersecretion of cortisol from the left adrenal mass and of aldosterone from the right one. A sequential bilateral laparoscopic adrenalectomy was performed. The cortisol level was normalized after partial left adrenalectomy and the aldosterone level was normalized after subsequent partial right adrenalectomy. Histopathological evaluation of the resected surgical specimens, including immunohistochemical staining for steroidogenic enzymes, revealed a left cortisol-producing adenoma and a right aldosterone-producing adenoma. The patient’s symptoms and laboratory findings resolved after sequential adrenalectomy without any pharmacological treatment.ConclusionsAdrenal venous sampling is essential in diagnosing bilateral functional adrenocortical adenomas prior to surgery. Proper interpretation of the laboratory findings is particularly important in these patients. Immunohistochemistry may be a valuable tool to identify aldosterone/cortisol-producing lesions and to validate the clinical diagnosis.
Highlights
Co-existing Cushing’s syndrome and primary aldosteronism caused by bilateral adrenocortical adenomas, secreting cortisol and aldosterone, respectively, have rarely been reported
Adrenal venous sampling is essential in diagnosing bilateral functional adrenocortical adenomas prior to surgery
The human adult adrenal cortex is composed of the zona glomerulosa (ZG), zona fasciculata (ZF), and zona reticularis (ZR), which are responsible for production of mineralocorticoids, glucocorticoids, and adrenal androgens, respectively [1]
Summary
We presented an extremely rare case of bilateral adrenal adenomas, the left of which secreted cortisol and the right of which secreted aldosterone, as determined by AVS and confirmed by histopathological evaluation of the resected surgical specimens, including immunohistochemical evaluation of steroidogenic enzymes. A/CPA, aldosterone- and cortisol-producing adenoma; APA, aldosteroneproducing adenoma; ARR, aldosterone-to-renin ratio; AVS, adrenal venous sampling; BPA, Bilateral partial adrenalectomy; CPA, cortisol-producing adenoma; CS, Cushing’s syndrome; HE, hematoxylin–eosin stain; IHC, Immunohistochemical; LPA, Left partial adrenalectomy; LTA, Left total adrenalectomy; NM, not mentioned; PAC, plasma aldosterone concentration; PRA, plasma renin activity; RPA, Right partial adrenalectomy; RTA, Right total adrenalectomy. Abbreviations 24 h UFC: Twenty-four-hour urine free cortisol; 3β-HSD: 3b-hydroxysteroid dehydrogenase; ACTH: Adrenocorticotropic hormone; APA: Aldosteroneproducing adenoma; ARR: Aldosterone-to-renin ratio; AV: Adrenal vein; AVS: Adrenal venous sampling; CPA: Cortisol-producing adenoma; CS: Cushing’s syndrome; CT: Computed tomography; CYP11B1: 11βhydroxylase; CYP11B2: 11beta-hydroxylase 2; HbA1c: glycosylated hemoglobin; IVC: Inferior vena cava; P450c17: 17alpha-hydroxylase; PA: Primary aldosteronism; PAC: Plasma aldosterone concentration; PRA: Plasma renin activity; SCS: Subclinical Cushing’s syndrome; ZF: Zona fasciculate; ZG: Zona glomerulosa; ZR: Zona reticularis
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