Aldosterone- and cortisol-co-producing adrenal adenoma without clinical features of Cushing syndrome

Chang, Kyung Yoon; Ryu, Seongyul; Cho, Jae-Young; Kim, Hyung Wook

doi:10.3904/kjim.2014.29.5.679

Cited by 5 publications

(4 citation statements)

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“…Both overt and subclinical PA and CS have long-term adverse effects on multiple organs [2, 3]. Patients with concurrent hypersecretion of cortisol and aldosterone have been previously reported, and it is difficult to make the correct diagnosis in those patients [4–8], however, most of these patients had adenomas that secreted both cortisol and aldosterone simultaneously, i.e. aldosterone- and cortisol-co-secreting adrenal tumors.…”

Section: Introductionmentioning

confidence: 99%

Hypercortisolism and primary aldosteronism caused by bilateral adrenocortical adenomas: a case report

et al. 2019

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Background Co-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 presentation We 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. Conclusions Adrenal 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. Electronic supplementary material The online version of this article (10.1186/s12902-019-0395-y) contains supplementary material, which is available to authorized users.

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Section: Introductionmentioning

confidence: 99%

Hypercortisolism and primary aldosteronism caused by bilateral adrenocortical adenomas: a case report

et al. 2019

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“…Subsequently, several cases of aldosterone-producing adenomas (APAs) with Cushing’s syndrome have been published [2–4]. APAs with concurrent subclinical cortisol hypersecretion (SCH) are also reported in an increasing number of patients [2, 5–9] characterized by a high cardiovascular risk [10], which reflects the combined damaging effects of the two steroid excess [11, 12]. In Japan, the prevalence of unilateral primary aldosteronism with concurrent subclinical hypercortisolism has been found to be 15 ~ 30% [6, 10].…”

Section: Introductionmentioning

confidence: 99%

Histopathological and genetic characterization of aldosterone-producing adenomas with concurrent subclinical cortisol hypersecretion: a case series

et al. 2017

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Purpose Aldosterone-producing adenomas with concurrent subclinical cortisol hypersecretion are reported in an increasing number of patients. Five aldosterone-producing adenomas from patients with primary aldosteronism and subclinical hypercortisolism were examined. The aims of our study were: (1) to analyze pathological features and immunohistochemical expression of CYP11B1 (11β-hydroxylase) and CYP11B2 (aldosterone synthase) in these tumors; (2) to investigate somatic mutations involved in adrenal steroid hypersecretion and/or tumor growth. Methods Archival micro-dissected paraffin-embedded slides from tumor specimens were used for histological and molecular studies. Immunohistochemistry was performed using monoclonal anti-CYP11B1 and anti-CYP11B2 antibodies. Cellular composition was determined by examining for known features of zona fasciculata and zona glomerulosa, and immunoreactivity for CYP11B1 and CYP11B2 by McCarty H-score. Spot regions for mutations in KCNJ5, ATP1A1, ATP2B3, CACNA1D, PRKACA, and CTNNB1 gene sequences were evaluated. Results Four APAs showed a predominant (≥50%) zona fasciculata-like cell pattern: one tumor had CYP11B1 H-score = 150, no detectable CYP11B2 expression, and harbored a PRKACA p.Leu206Arg mutation (that we have reported previously elsewhere), one had no CYP11B1 expression, CYP11B2 H-score = 40, and no mutations; the remaining two adenomas had high CYP11B1 H-score (160 and 240, respectively) and low CYP11B2 H-score (30 and 15, respectively), with the latter harboring a CTNNB1 p. Ser45Phe activating mutation. One of five aldosterone-producing adenomas had a predominant zona glomerulosa-like pattern, CYP11B1 H-score = 15, CYP11B2 H-score = 180, and no mutations. Conclusions The majority of aldosterone-producing adenomas with concurrent subclinical cortisol hypersecretion were composed mainly of zona fasciculata-like cells, while CYP11B1 and CYP11B2 immunostaining demonstrated clear heterogeneity. In a subset of cases, different somatic mutations may be involved in hormone excess and tumor formation.

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“…Most of these patients had adenomas that secreted both cortisol and aldosterone simultaneously, i.e., aldosterone- and cortisol-cosecreting adrenal tumors ( 7 – 9 ). However, it is difficult to make the correct diagnosis in patients with concurrent PA and SCS ( 10 ). Thus, we present a case of coexistent PA and SCS to summarize the diagnosis and management of this disorder.…”

Section: Introductionmentioning

confidence: 99%

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

Ji²,

Tao³

et al. 2022

Front. Cardiovasc. Med.

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BackgroundCoexisting primary aldosteronism (PA) and subclinical Cushing's syndrome (SCS) caused by bilateral adrenocortical adenomas have occasionally been reported. Precise diagnosis and treatment of the disease pose a challenge to clinicians due to its atypical clinical manifestations and laboratory findings.Case SummaryA 49-year-old woman was admitted to our hospital due to fatigue, increased nocturia and refractory hypertension. The patient had a history of severe left hydronephrosis 6 months prior. Laboratory examinations showed hypokalaemia (2.58 mmol/L) and high urine potassium (71 mmol/24 h). Adrenal computed tomography (CT) showed bilateral adrenal masses. Undetectable ACTH and unsuppressed plasma cortisol levels by dexamethasone indicated ACTH-independent Cushing's syndrome. Although the upright aldosterone-to-renin ratio (ARR) was 3.06 which did not exceed 3.7, elevated plasma aldosterone concentrations (PAC) with unsuppressed PAC after the captopril test still suggested PA. Adrenal venous sampling (AVS) without adrenocorticotropic hormone further revealed hypersecretion of aldosterone from the right side and no dominant side of cortisol secretion. A laparoscopic right adrenal tumor resection was performed. The pathological diagnosis was adrenocortical adenoma. After the operation, the supine and standing PAC were normalized; while the plasma cortisol levels postoperatively were still high and plasma renin was activated. The patient's postoperative serum potassium and 24-h urine potassium returned to normal without any pharmacological treatment. In addition, the patient's blood pressure was controlled normally with irbesartan alone.ConclusionPatients with refractory hypertension should be screened for the cause of secondary hypertension. AVS should be performed in patients in which PA is highly suspected to determine whether there is the option of surgical treatment. Moreover, patients with PA should be screened for hypercortisolism, which can contribute to a proper understanding of the AVS result.

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Aldosterone- and cortisol-co-producing adrenal adenoma without clinical features of Cushing syndrome

Cited by 5 publications

References 5 publications

Hypercortisolism and primary aldosteronism caused by bilateral adrenocortical adenomas: a case report

Hypercortisolism and primary aldosteronism caused by bilateral adrenocortical adenomas: a case report

Histopathological and genetic characterization of aldosterone-producing adenomas with concurrent subclinical cortisol hypersecretion: a case series

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

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