Aldosterone-secreting Adenoma Research Articles

PRIMARY ALDOSTERONISM: THE EFFECT OF SPECIFIC TREATMENT ON RENAL FUNCTION

Objective: The aim of the study was to evaluate the changes in eGFR in patients with idiopathic primary aldosteronism (IHA) and with aldosterone-secreting adenoma (APA) after specific therapy. Design and method: From January 2018 to March 2022, we consecutively enrolled 99 patients with PA distinguishing 75 with IHA (mean age 53.41±11.97 years) and 24 with PA (average age 48.79±8.96 years). We evaluated anthropometric, biochemicals and hemodynamic parameters at diagnosis and 12 months after pharmacological or surgical therapy. Results: Patients with IHA, pharmacologically treated with mineralocorticoid receptor antagonists (MRAs), showed a higher incidence of chronic renal failure and a faster progression of renal damage than in patients with APA who underwent unilateral adrenalectomy. The reduction of eGFR was significantly greater in patients with IHA than in APA (92.03±17.58 vs 86.63±18.89 mL/min/1.73m2, p <0.001). Systolic blood pressure (SBP) levels and increased aldosterone-to-renin ratio were found to be independent predictors of renal function decline (ß = 0.034; p = 0.045 and ß = 0.248; p = 0.032, respectively). Conclusions: Our results show that patients with PA undergoing surgical treatment have a better renal outcome than those receiving drug therapy. An early diagnosis of APA would allow intervention before renal damage occurs.

Intraprocedural cortisol testing improves adrenal vein cannulation success and diagnostic accuracy in assessment of primary aldosteronism, in a medium throughput centre

Primary aldosteronism is the most common cause of secondary hypertension. Identifying individuals who have unilateral secretion from aldosterone secreting adenomas allows adrenalectomy. Surgical treatment when feasible may be superior to medical management with improved cardiovascular outcomes and reduced medication dependence. Adrenal vein sampling (AVS) is required to biochemically lateralise aldosterone secretion prior to adrenalectomy. However, diagnostic success of AVS is variable and can be poor even at tertiary centres; failure is largely due to unsuccessful adrenal vein cannulation. Intra-procedural rapid semiquantitative cortisol testing (RCT) identifies correct catheter placement in real time. We compared diagnostic success rates of AVS before and after the introduction of intraprocedural cortisol testing at the Royal Adelaide Hospital—a medium throughput tertiary centre (average 6.2 procedures a year over the last 8 years). We observed an increase in success rate from 63% to 94%. Intraprocedural cortisol testing also led to a net financial saving of ~$100 AUD per procedure. RCT is likely to be cost effective if pre-RCT success rate is less than 78%. Procedure time and number of samples collected, however, were increased with RCT. This suggests that intraprocedural cortisol testing will improve success in low to medium throughput centres and may make AVS feasible in less specialised centres.

Is the Adrenal Incidentaloma Functionally Active? An Approach-To-The-Patient-Based Review.

Adrenal incidentalomas are a common occurrence. Most of them are adrenocortical adenomas that do not cause harm and do not require surgery, but a non-negligible proportion of incidentalomas is represented by functionally active masses, including cortisol-secreting adenomas (12%), pheochromocytomas (3–6%), aldosterone-secreting adenomas (2–3%), as well as malignant nodules, such as adrenocortical carcinomas (2–5%), which can be either functioning or non-functioning. All patients with an adrenal incidentaloma should undergo a few biochemical screening and confirmatory tests to exclude the presence of a functionally active mass. In this approach-to-the-patient-based review, we will summarize current recommendations on biochemical evaluation and management of functionally active adrenal incidentalomas. For this purpose, we will present four case vignettes, whereby we will describe how patients were managed, then we will review and discuss additional considerations tied to the diagnostic approach, and conclude with practical aspects of patient perioperative management. To improve the perioperative management of patients with functional adrenal incidentalomas, multidisciplinary meetings are advocated.

The role of surgery in Conn's syndrome -a case of refractory hypertension secondary to an aldosterone secreting adenoma

SUMMARY Primary aldosteronism, eponymously known as Conn's syndrome, has recently gained recognition as the most common cause of endocrine hypertension. Unilateral subtypes, such as aldosterone secreting adenomas, are managed surgically and are potentially curable. The background of refractory hypertension and hypokalaemia in a forty-year-old man raised suspicions of Conn's syndrome which was localised to an aldosterone secreting adenoma in the right adrenal gland. The patient underwent a laparoscopic intraperitoneal right adrenalectomy which resulted in normalisation of his plasma aldosterone concentration as well as improved blood pressure control.

The Invisible Evil Twin of an Adrenal Adenoma

Primary aldosteronism (PA) causes a persistently elevated blood pressure (BP) due to excessive release of the hormone aldosterone from the adrenal glands. Classically, it is called Conn’s syndrome and is described as the triad of hypertension and hypokalemia with the presence of unilateral adrenal adenoma. It can be cured with surgical resection of the aldosterone-secreting adenoma leading to resolution of hypertension, hypokalemia and increased cardiovascular risk associated with hyperaldosteronism. We present a case of a man with previous ischemic heart disease (IHD) who presented with resistant hypertension. Investigations for secondary causes of hypertension revealed an elevated aldosterone level and saline suppression test confirmed the diagnosis of PA. Radiological examination revealed a left adrenal adenoma and a normal right adrenal gland. However, adrenal venous sampling showed lateralization of aldosterone secretion towards the right. He subsequently underwent a laparoscopic right adrenalectomy which improved his BP control promptly. This case highlights the importance of recognizing the need to investigate for secondary causes of hypertension. It also underscores the importance of dynamic tests, which may not be easily accessible to most clinicians but should pursue, to allow a definitive diagnosis and effective treatment.

Diagnostic efficacy of 18F-FDG PET/CT in patients with adrenal incidentaloma.

BackgroundThe management of adrenal incidentaloma is still a challenge with respect to determining its functionality (hormone secretion) and malignancy. In this light, we performed 18F-FDG PET/CT scan to assess the SUVmax values in different adrenal masses including Cushing syndrome, pheochromocytoma, primary hyperaldosteronism and non-functional adrenal adenomas.MethodsTotal 109 (73 F, 36 M) patients with adrenal mass (incidentaloma), mean age of 53.3 ± 10.2 years (range, 24–70) were screened by 18F-FDG PET/CT. Data of 18F-FDG PET/CT imaging of the patients were assessed by the same specialist. Adrenal masses were identified according to the calculated standardized uptake values (SUVs). Clinical examination, 24-h urine cortisol, catecholamine metabolites, 1-mg dexamethasone suppression test, aldosterone/renin ratio and serum electrolytes were analyzed.ResultsBased on the clinical and hormonal evaluations, there were 100 patients with non-functional adrenal mass, four with cortisol-secreting, four with pheochromocytomas and one with aldosterone-secreting adenoma. Mean adrenal mass diameter of 109 patients was 2.1 ± 4.3 (range, 1–6.5 cm). The 18F-FDG PET/CT imaging of the patients revealed that lower SUVmax values were found in non-functional adrenal masses (SUVmax 3.2) when compared to the functional adrenal masses including four with cortisol-secreting adenoma (SUVmax 10.1); four with pheochromcytoma (SUVmax 8.7) and one with aldosterone-secreting adenomas (SUVmax 3.30). Cortisol-secreting (Cushing syndrome) adrenal masses showed the highest SUVmax value (10.1), and a cut-off SUVmax of 4.135 was found with an 84.6% sensitivity and 75.6% specificity cortisol-secreting adrenal adenoma.ConclusionsConsistent with the similar studies, non-functional adrenal adenomas typically do not show increased FDG uptake and a certain form of functional adenoma could present various FDG uptake in FDG PET/CT. Especially functional adrenal adenomas (cortisol secreting was the highest) showed increased FDG uptake in comparison to the non-functional adrenal masses. Therefore, setting a specific SUVmax value in the differentiation of malignant adrenal lesion from the benign one is risky and further studies, including a high number of functional adrenal mass are needed.

Evidence of aldosterone synthesis in human myocardium in acute myocarditis

BackgroundMyocarditis may be self-limited but has been identified as an important contributor to downstream cardiomyopathy. Aldosterone mediates myocardial damage in various conditions, but has not been considered specifically as a therapeutic target for inflammatory damage in acute myocarditis. We sought to demonstrate local aldosterone synthesis in human myocardium affected by acute myocarditis. MethodsWe evaluated myocardial samples obtained via endomyocardial biopsy (EMB) for expression of CYP11B2, the final and key enzyme for aldosterone synthesis, from patients with acute myocarditis and from stable heart transplant recipients with no evidence of rejection as negative controls. Excised adrenal glands from patients with aldosterone-secreting adenomas were used as positive controls. An experienced cardiovascular pathologist blinded to clinical information rated CYP11B2 stains as negative, positive, or borderline, also recording location of any CYP11B2-positivity. ResultsSixteen patients' EMB samples showing definite acute myocarditis were identified (50% female). CYP11B2 was positive in 13/16 cases (81%), typically showing diffuse intracardiomyocyte cytoplasmic staining, vs. 2/16 borderline stains in transplant controls (p < 0.001 myocarditis vs. negative controls). All 3 adrenalectomy samples stained positive for CYP11B2 (diffuse intracellular staining). Importantly, no myocarditis or transplant patients were on aldosterone antagonist therapy at the time of biopsy. ConclusionsIn this proof-of-concept study, myocardium from patients with acute myocarditis demonstrates evidence and high prevalence of local aldosterone synthesis by immunohistochemistry that showed high accuracy, specificity, and sensitivity. Aldosterone warrants consideration as a specific target for therapy in patients with myocardial damage due to inflammation towards strategies that reduce downstream complications.

Who was Dr. William C. Baum?

The first discovery of primary hyperaldosteronism secondary to an aldosterone-secreting adrenal adenoma has been credited solely to Dr. Jerome Conn, an endocrinologist at the University of Michigan and for whom, Conn syndrome was named. Dr. William Baum, a urologist at the University of Michigan, however, was instrumental in the appropriate operation and historical aldosteronoma resection. Despite Dr. Baum's important role in this discovery, he was never included as an author in any of the subsequent papers describing Conn syndrome and, few today would recognize his name. So, who was Dr. Baum and what happened? This historical article aims to revisit the history surrounding the discovery of aldosteronoma as a cause of Conn's syndrome and to catalog the life and involvement of Dr. William C. Baum in that discovery.

Pure aldosterone-secreting adrenocortical carcinoma in a patient with refractory primary hyperaldosteronism.

SummaryWe describe a young male patient with longstanding hypertension, who was diagnosed with primary hyperaldosteronism and treated by an attempted retroperitoneoscopic total unilateral adrenalectomy for a left-sided presumed aldosterone-secreting adenoma. Imaging had shown an unremarkable focal adrenal lesion with normal contralateral adrenal morphology, and histology of the resected specimen showed no adverse features. Post-operatively, his blood pressure and serum aldosterone levels fell to the normal range, but 9 months later, his hypertension recurred, primary aldosteronism was again confirmed and he was referred to our centre. Repeat imaging demonstrated an irregular left-sided adrenal lesion with normal contralateral gland appearances. Adrenal venous sampling was performed, which supported unilateral (left-sided) aldosterone hypersecretion. Redo surgery via a laparoscopically assisted transperitoneal approach was performed and multiple nodules were noted extending into the retroperitoneum. It was thought unlikely that complete resection had been achieved. His blood pressure returned to normal post-operatively, although hypokalaemia persisted. Histological examination, from this second operation, showed features of an adrenocortical carcinoma (ACC; including increased mitoses and invasion of fat) that was assessed as malignant using the scoring systems of Weiss and Aubert. Biochemical hyperaldosteronism persisted post-operatively, and detailed urine steroid profiling showed no evidence of adrenal steroid precursors or other mineralocorticoid production. He received flank radiotherapy to the left adrenal bed and continues to receive adjunctive mitotane therapy for a diagnosis of a pure aldosterone-secreting ACC. Learning points Pure aldosterone-secreting ACCs are exceptionally uncommon, but should be considered in the differential diagnosis of patients presenting with primary aldosteronism.Aldosterone-producing ACCs may not necessarily show typical radiological features consistent with malignancy.Patients who undergo surgical treatment for primary aldosteronism should have follow-up measurements of blood pressure to monitor for disease recurrence, even if post-operative normotension is thought to indicate a surgical ‘cure’.Owing to the rarity of such conditions, a greater understanding of their natural history is likely to come from wider cooperation with, and contribution to, large multi-centre outcomes databases.

4:24 PM, Abstract No. 312 - Adrenal vein sampling: a simple method to increase the technical success rate

Adrenal vein sampling is considered the gold standard for distinguishing unilateral aldosterone-secreting adenomas from bilateral adrenal hyperplasia, but has a reputation as a difficult procedure. Our institution employs obtaining samples from multiple sites in the region of the adrenal veins to ensure that the adrenal vein is sampled. The purpose of this study is to show that increasing the number of vessels sampled increases efficacy without significantly increasing cost.

Hypertension artérielle d’origine surrénale : reconnaître, évaluer et prendre en charge

The frequency of the diagnosed causes of secondary hypertension is only known from hospital-based records, which probably overestimate the true prevalence. Excluding oral contraceptive users and cases with renal failure, their overall frequency was estimated at 1 percent in the eighties, 5 percent in the nineties, and 9 percent in recent years. This increase in frequency was mostly due to an increased number of diagnosed cases of endocrine hypertension. The diagnosis of endocrine hypertension is not synonymous with the diagnosis of a surgically correctable form of hypertension. Indeed, hypertension is surgically curable in a minority of patients, mostly in patients with aldosterone-secreting adenomas or with pheochromocytomas or functional paragangliomas. The presentation, screening, diagnosis and therapeutic management of endocrine hypertension are discussed in the present issue.

Clinical Characteristics and Metabolic Features of Patients with Adrenal Incidentalomas with or without Subclinical Cushing's Syndrome.

BackgroundThe aim of this study was to examine the clinical characteristics of adrenal incidentalomas discovered by computed tomography (CT) and to investigate metabolic features of subclinical Cushing's syndrome (SCS) in patients with adrenal incidentalomas in a tertiary hospital in Korea.MethodsThis retrospective study examined the clinical aspects of 268 patients with adrenal incidentalomas discovered by CT at Soonchunhyang University Bucheon Hospital. Clinical data and endocrine function of the patients as well as histological findings were obtained from medical records, while anatomic characteristics were analyzed by reviewing imaging studies. Hormonal tests for pheochromocytoma, Cushing's syndrome, and aldosterone-secreting adenoma were performed.ResultsMost (n=218, 81.3%) cases were nonfunctioning tumors. Of the 50 patients with functioning tumors (18.7%), 19 (7.1%) were diagnosed with SCS, nine (3.4%) with overt Cushing's syndrome, 12 (4.5%) with primary aldosteronism, and 10 (3.7%) with pheochromocytoma. Malignant tumors (both primary and metastatic) were rare (n=2, 0.7%). Body mass index, fasting glucose, hemoglobin A1c, and total cholesterol were significantly higher in patients with SCS in comparison with those with nonfunctioning tumors. The prevalence of type 2 diabetes mellitus and hypertension were significantly higher in patients with SCS compared with those with nonfunctioning tumors.ConclusionFunctioning tumors, especially those with subclinical cortisol excess, are commonly found in patients with adrenal incidentalomas, although malignancy is rare. In addition, patients with SCS in adrenal incidentalomas have adverse metabolic and cardiovascular profiles.

Management of hypertension in primary aldosteronism.

Hypertension causes significant morbidity and mortality worldwide, owing to its deleterious effects on the cardiovascular and renal systems. Primary hyperaldosteronism (PA) is the most common cause of reversible hypertension, affecting 5%-18% of adults with hypertension. PA is estimated to result from bilateral adrenal hyperplasia in two-thirds of patients, and from unilateral aldosterone-secreting adenoma in approximately one-third. Suspected cases are initially screened by measurement of the plasma aldosterone-renin-ratio, and may be confirmed by additional noninvasive tests. Localization of aldostosterone hypersecretion is then determined by computed tomography imaging, and in selective cases with adrenal vein sampling. Solitary adenomas are managed by laparoscopic or robotic resection, while bilateral hyperplasia is treated with mineralocorticoid antagonists. Biochemical cure following adrenalectomy occurs in 99% of patients, and hemodynamic improvement is seen in over 90%, prompting a reduction in quantity of anti-hypertensive medications in most patients. End-organ damage secondary to hypertension and excess aldosterone is significantly improved by both surgical and medical treatment, as manifested by decreased left ventricular hypertrophy, arterial stiffness, and proteinuria, highlighting the importance of proper diagnosis and treatment of primary hyperaldosteronism. Although numerous independent predictors of resolution of hypertension after adrenalectomy for unilateral adenomas have been described, the Aldosteronoma Resolution Score is a validated multifactorial model convenient for use in daily clinical practice.

Adrenal lesions in patients of multiple endocrine neoplasia type 1

To investigate the prevalence and characteristics of adrenal lesions in Chinese multiple endocrine neoplasia type 1 (MEN-1) patients. Adrenal CT scan and clinical manifestations were retrospectively reviewed in 32 consecutive MEN-1 patients who were evaluated at our hospital during January 1986 to December 2009. Adrenal lesions were identified in 16 of 32 (50%) MEN-1 patients. Five (31.3%) patents with adrenal involvement showed bilateral lesions, including bilateral adenoma (n=1), bilateral hyperplasia (n=2) and adenoma and hyperplasia on each side (n=2). Unilateral adrenal lesion was presented in 11 (68.7%) patients. Among which, 63.6% had adenomas with a mean diameter of 2.3 cm (0.8-4.0 cm) and the remainder was of hyperplasia or enlargement. In two patients, functioning adrenal abnormalities were detected including Cushing adenoma (n=1) and aldosterone-secreting adenoma (n=1). The prevalence of adrenal lesion in MEN-1 patient is similar between China and western countries. These tumors are mostly benign, small and nonfunctioning. Taking into account a high incidence of adrenal carcinoma in previous foreign studies, routine screening and close surveillance are still recommended for adrenal lesions in MEN-1 patients.

Adrenomedullin 2/Intermedin in the Hypothalamo–Pituitary–Adrenal Axis

Adrenomedullin 2/intermedin (AM2/IMD) is a new member of the calcitonin/calcitonin gene-related peptide (CGRP) family. CGRP, adrenomedullin (AM), and AM2/IMD share the receptor system consisting of calcitonin receptor-like receptor (CRLR) and receptor activity-modifying proteins (RAMP). The CRLR/RAMP2 or CRLR/RAMP3 complex forms the AM receptor, whereas the CRLR/RAMP1 forms the CGRP receptor. AM2/IMD binds non-selectively to all three CRLR/RAMP complexes. AM2/IMD has various actions, such as a potent vasodilator action and a protective action against oxidative stress, like AM and CGRP. When administered intracerebroventricularly, AM2/IMD stimulates the sympathetic nervous system and increases blood pressure. In human hypothalamus, AM2/IMD is expressed in the paraventricular and supraoptic nuclei and colocalized with arginine vasopressin. Anterior pituitary cells were diffusely immunostained for AM2/IMD. AM2/IMD stimulates the release of ACTH, prolactin, and oxytocin, but suppresses GH release. Some of these pituitary actions of AM2/IMD have been supposed to be mediated by an unidentified unique receptor for AM2/IMD. In the adrenal gland, immunoreactive (IR)-AM2/IMD and IR-AM were detected in the medulla, while the degree of IR-AM2/IMD and IR-AM in the cortex was relatively weak or undetectable. Furthermore, AM2/IMD and AM were expressed in adrenocortical tumors, such as aldosterone-secreting adenomas, and pheochromocytomas. CRLR and RAMPs are expressed in the hypothalamus, pituitaries, adrenal glands, and adrenal tumors. Thus, AM2/IMD is expressed in every endocrine organ of the hypothalamo-pituitary-adrenal axis together with its receptor. AM2/IMD may act as a neurotransmitter or modulator in the brain and as a paracrine/autocrine regulator in the hypothalamo-pituitary-adrenal axis.

Diagnosis and management of primary aldosteronism

Purpose of review To illustrate the steps for clinical management of primary aldosteronism from screening evaluation to surgical and/or medical treatment. Recent findings It is now widely accepted that primary aldosteronism represents the most common form of endocrine hypertension and its early diagnosis is crucial for hypertensive patients who can be cured by the surgical removal of an aldosterone-secreting adenoma or benefit from a specific medical treatment with mineralocorticoid receptor antagonists. Recent evidence indicates that hyperaldosteronism is indeed associated with detrimental consequences on cardiovascular system, renal function and glucose metabolism. Summary The diagnostic protocol for primary aldosteronism requires multistep evaluation and should begin with a screening test, followed when appropriate, by confirmatory test and functional and anatomical evaluation. Finally, although it is technically difficult and the cut-off levels for acceptance of the success are not standardized, the subtype forms should be identified using a selective adrenal venous sampling.

Diagnosis and management of primary aldosteronism

To illustrate the steps for clinical management of primary aldosteronism from screening evaluation to surgical and/or medical treatment. It is now widely accepted that primary aldosteronism represents the most common form of endocrine hypertension and its early diagnosis is crucial for hypertensive patients who can be cured by the surgical removal of an aldosterone-secreting adenoma or benefit from a specific medical treatment with mineralocorticoid receptor antagonists. Recent evidence indicates that hyperaldosteronism is indeed associated with detrimental consequences on cardiovascular system, renal function and glucose metabolism. The diagnostic protocol for primary aldosteronism requires multistep evaluation and should begin with a screening test, followed when appropriate, by confirmatory test and functional and anatomical evaluation. Finally, although it is technically difficult and the cut-off levels for acceptance of the success are not standardized, the subtype forms should be identified using a selective adrenal venous sampling.

Differential Expression of Ghrelin and its Receptor (GHS-R1a) in Various Adrenal Tumors and Normal Adrenal Gland

Ghrelin is a newly characterized, widely distributed peptide thought to be involved in the regulation of appetite. Significant effects on the release of growth hormone (GH) and ACTH have been demonstrated. This study compares the expression of ghrelin and its receptor (GHS-R) in various adrenal tumors and normal adrenal gland. Normal adrenal tissue was obtained after autopsy. Tissue was obtained from 13 pheochromocytomas (PHEOs), 15 cortisol-secreting adenomas (CPAs), 12 aldosterone-secreting adenomas (APAs), and 16 nonfunctional adenomas (NFAs) following laparoscopic surgery. Expression of ghrelin and GHS-R1a was investigated on RNA levels by using real-time reverse transcription polymerase chain reaction (RT-PCR) and on protein levels by using immunohistochemistry. In the seven normal adrenal glands analyzed, ghrelin mRNA levels were 12-fold lower than in stomach. Ghrelin protein expression was confirmed by immunohistochemistry. In all adrenal tumors, relevant levels of ghrelin mRNA were observed, with significantly lower expression in PHEOs and APAs than in normal adrenal gland. Ghrelin protein was detected in 0% of PHEOs, 55% of APAs, 87% of CPAs, and 54% of NFAs. GHS-R1a mRNA expression was detectable in normal adrenal gland, but the receptor protein was absent. In adrenal tumors, detectable levels of receptor mRNA were found in 38% of PHEOs, 13% of CPAs, and 25% of NFAs. GHS-R1a protein was absent in the majority of adrenal tumors. Expression of ghrelin in normal adrenal gland and adrenal tumors may indicate some unknown physiological function. The pathophysiological relevance of ghrelin expression in adrenal tumors remains to be investigated.

Immunohistochemical localization of somatostatin receptor subtypes in benign and malignant adrenal tumours

Somatostatin mediates its action through five receptor subtypes (sst1-5) that are widely distributed in various endocrine tissues and tumours. Because of the inhibitory effects of somatostatin, long-acting analogues have been synthesized. In contrast to their well-established use in neuroendocrine and pituitary tumours, little is known about their potential use in adrenal tumours. We examined somatostatin receptor protein expression in adrenal tumours of various aetiologies. Immunostaining was performed with specific polyclonal antibodies for sst1-5. Seven benign and eight malignant pheochromocytomas (PHEOs), eight aldosterone-secreting adenomas (APAs), nine cortisol-secreting adenomas (CPAs), seven nonfunctioning adrenal tumours (NFAs) and 25 adrenal carcinomas (CAs) as well as eight normal adrenal glands were investigated. Staining pattern, distribution and subcellular localization of the somatostatin receptor subtypes were evaluated. In the majority of normal cortices the expression of sst1-5 was limited to the reticular zone. The medulla was predominantly positive for sst3. Most cortical adenomas were positive for all five subtypes. However, in the majority of these tumours, less than 30% of cells were positively stained. A high expression of sst4 was found in CPAs but only very few cortical carcinomas exhibited sst immunostaining. All benign PHEOs were positive for sst3. The majority presented with more than 60% of tumour cells stained. By contrast, only six out of eight malignant PHEOs were positive for sst3. Somatostatin receptor subtypes are expressed in PHEOs as well as in tumours of the adrenal cortex with tumour-specific distribution patterns. This may offer new diagnostic and therapeutic possibilities.

An additional child case of an aldosterone-producing adenoma with an atypical presentation of peripheral paralysis due to hypokalemia

Aldosterone-producing adenoma, which is characterized by hypertension, hypokalemia, and elevated aldosterone levels with suppressed plasma renin activity, is a rare condition during childhood and is also potentially curable. To the best of our knowledge, nearly 25 cases of childhood aldosterone-secreting adenoma have been reported in the literature to date. Here we describe a 13-yr-old girl with primary hyperaldosteronism secondary to aldosterone-secreting adenoma. The patient was admitted to our hospital with the neuromuscular complaints of muscle weakness and inability to walk due to hypokalemia. She had been misdiagnosed as having hypokalemic periodic paralysis 2 months before admission and her symptoms had radically improved with potassium supplementation. However, her blood pressure levels had increased and her symptoms reappeared 2 days prior to being observed during hospitalization in our institution. Laboratory examinations revealed hypokalemia (2.1 mEq/l), and increased serum aldosterone levels with suppressed plasma renin activity. Abdominal ultrasonography and abdominal magnetic resonance imaging revealed left adrenal mass. Laparoscopic adrenalectomy was performed and histopathological examinations showed benign adrenal adenoma. Serum aldosterone levels and blood pressure levels returned to normal after surgical intervention. This case demonstrates the importance of a systemic evaluation including blood pressure monitorization of children with hypokalemia as intermittent hypertension episodes may be seen; cases without hypertension may be misdiagnosed as rheumatological or neurological disorders such as hypokalemic periodic paralysis, as in our case.