Primary aldosteronism concurrent with subclinical Cushing’s syndrome: a case report and review of the literature

Abstract

Background: The prevalence of primary aldosteronism concurrent with subclinical Cushing's syndrome was higher than previously thought. Through analyzing a rare clinical case, we summarized the diagnosis and management of primary aldosteronism with subclinical Cushing's syndrome. Case presentation: A 54-year-old Chinese man of Han nationality was diagnosed as having primary aldosteronism with subclinical Cushing's syndrome. An abdominal computed tomography scan revealed a mass in his left adrenal gland and a mass in his right adrenal gland. After finishing sequential adrenal venous sampling without adrenocorticotropic hormone, the result reminded us that the left and right nodules were responsible for hypercortisolism and aldosterone hypersecretion, respectively. Right and left adrenalectomy were performed successively. The pathological diagnosis was adrenocortical adenoma for both. Histological findings revealed that the right one had positive immunostaining for CYP11B2 and the left one had positive immunostaining for CYP11B1. The immunohistochemistry result helped us to confirm the diagnosis. Somatic KCNJ5 mutation (Leu168Arg) was found in the right tumor; there was no KCNJ5 mutation in the left adrenal tumor. Conclusions: We suggest that patients with primary aldosteronism should have a low-dose overnight dexamethasone suppression test to screen for hypercortisolism. It can help avoid misdiagnoses and contribute to proper understanding of the adrenal vein sampling result. Making sure of the nidus of aldosterone and cortisol secretion is crucial for the therapy of patients with primary aldosteronism and subclinical Cushing's syndrome.

Case Report: Primary Aldosteronism and Subclinical Cushing Syndrome in a 49-Year-Old Woman With Hypertension Plus Hypokalaemia

“…They measured the concentrations of plasma epinephrine in both AV and IVC to enable adjustment in the present patient. Yingxiao Zhang et al ( 22 ) reported a PA and SCS case of bilateral adrenal tumors, in which the left adrenocortical tumor produced cortisol and the right one secreted aldosterone. The diagnosis was supported by endocrine findings, AVS and immunohistochemical evaluation of steroidogenic enzymes.…”

“…SCS is generally found when a patient with an unidentified adrenal mass underwent DMST, and it is the most common endocrine syndrome associated with an adrenal adenoma ( 22 , 24 ). Many cases of PA with SCS have been reported as aldosterone/cortisol cosecreting adenomas ( 6 ).…”

Case Report: Primary Aldosteronism Due to Bilateral Aldosterone-Producing Micronodules With HISTALDO Classical and Contralateral Non-Classical Pathology

“…A recent report from Zhang et al. raised the concern that PA patients should have low-dose overnight DST to rule out the existence of concurrent ACS and to avoid misinterpretations of AVS results that may lead to suboptimal managements of the patients postoperatively due to the emergence of temporary hypocortisonism in patients with preoperative concurrent ACS ( 27 ). Given the significant prevalence of uPA patients with cortisol co-secretion, our evidence also supports the practice that all uPA patients should have their baseline 1 mg DST measured to not only help predict surgical outcomes, but to also inform suitable postoperative steroid supplement and tapering regimens for the prevention of adrenal insufficiency.…”

Hypertension with unilateral adrenal aldosterone and cortisol cosecreting adenoma: A case report

“…Primary aldosteronism with subclinical Cushing's syndrome used to be thought as a rare disease 1,2 . However, with the recent substantial increase in the diagnosis of primary aldosteronism, there was an increase in the reporting of cases with the coexistence of primary aldosteronism and subclinical Cushing's syndrome 3,4,5,6,7 . Although subclinical Cushing's syndrome often has no clinical manifestations, its coexistence with primary aldosteronism is clinically relevant for the diagnosis and treatment of the latter disease.…”

“… 1 , 2 However, with the recent substantial increase in the diagnosis of primary aldosteronism, there was an increase in the reporting of cases with the coexistence of primary aldosteronism and subclinical Cushing's syndrome. 3 , 4 , 5 , 6 , 7 Although subclinical Cushing's syndrome often has no clinical manifestations, its coexistence with primary aldosteronism is clinically relevant for the diagnosis and treatment of the latter disease. The lateralization of primary aldosteronism relies on the contrast of the aldosterone and cortisol concentrations.…”