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

2015 ◽  
Vol 2015 ◽  
Author(s):  
S Hussain ◽  
E Panteliou ◽  
D M Berney ◽  
R Carpenter ◽  
M Matson ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Adrenocortical Carcinoma ◽  
Primary Aldosteronism ◽  
Young Male ◽  
Scoring Systems ◽  
Primary Hyperaldosteronism ◽  
Adrenal Lesion ◽  
Resected Specimen ◽  
List Type ◽  
Unilateral Adrenalectomy

Summary We 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.

scholarly journals KCNJ5 Somatic Mutation Is a Predictor of Hypertension Remission After Adrenalectomy for Unilateral Primary Aldosteronism

2019 ◽  
Vol 104 (10) ◽  
pp. 4695-4702 ◽  
Author(s):  
Leticia A P Vilela ◽  
Marcela Rassi-Cruz ◽  
Augusto G Guimaraes ◽  
Caio C S Moises ◽  
Thais C Freitas ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Somatic Mutation ◽  
Primary Aldosteronism ◽  
Antihypertensive Drugs ◽  
Independent Predictor ◽  
Blood Pressure Response ◽  
Antihypertensive Medications ◽  
Unilateral Adrenalectomy ◽  
Molecular Features ◽  
Endocrine Hypertension

AbstractContextPrimary aldosteronism (PA) is the most common cause of endocrine hypertension (HT). HT remission (defined as blood pressure <140/90 mm Hg without antihypertensive drugs) has been reported in approximately 50% of patients with unilateral PA after adrenalectomy. HT duration and severity are predictors of blood pressure response, but the prognostic role of somatic KCNJ5 mutations is unclear.ObjectiveTo determine clinical and molecular features associated with HT remission after adrenalectomy in patients with unilateral PA.MethodsWe retrospectively evaluated 100 patients with PA (60 women; median age at diagnosis 48 years with a median follow-up of 26 months). Anatomopathological analysis revealed 90 aldosterone-producing adenomas, 1 carcinoma, and 9 unilateral adrenal hyperplasias. All patients had biochemical cure after unilateral adrenalectomy. KCNJ5 gene was sequenced in 76 cases.ResultsKCNJ5 mutations were identified in 33 of 76 (43.4%) tumors: p.Gly151Arg (n = 17), p.Leu168Arg (n = 15), and p.Glu145Gln (n = 1). HT remission was reported in 37 of 100 (37%) patients. Among patients with HT remission, 73% were women (P = 0.04), 48.6% used more than three antihypertensive medications (P = 0.0001), and 64.9% had HT duration <10 years (P = 0.0015) compared with those without HT remission. Somatic KCNJ5 mutations were associated with female sex (P = 0.004), larger nodules (P = 0.001), and HT remission (P = 0.0001). In multivariate analysis, only a somatic KCNJ5 mutation was an independent predictor of HT remission after adrenalectomy (P = 0.004).ConclusionThe presence of a KCNJ5 somatic mutation is an independent predictor of HT remission after unilateral adrenalectomy in patients with unilateral PA.

scholarly journals Testicular swelling leading to a diagnosis of adrenocortical carcinoma – co-incidence or causation?

2021 ◽  
Vol 2021 ◽  
Author(s):  
Maria Batool ◽  
David Fennell ◽  
David Slattery ◽  
Eamon Leen ◽  
Liam Cormican ◽  
...  
Keyword(s):  
Histological Examination ◽  
Adrenocortical Carcinoma ◽  
Steroid Hormone ◽  
Abdominal Mass ◽  
Resected Specimen ◽  
List Type ◽  
Caucasian Male ◽  
Potential Link ◽  
Rare Malignancy ◽  
Learning Points

Summary Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million/year. A majority of patients present with steroid hormone excess or abdominal mass effects, and in 15% of patients ACC is discovered incidentally. We present a case of 30-year-old otherwise asymptomatic Caucasian male who presented with a testicular swelling. Subsequent imaging and investigations revealed disseminated sarcoidosis and an 11 cm adrenal lesion. An adrenalectomy was performed. Histological examination of the resected specimen confirmed an ACC and also demonstrated a thin rim of adrenal tissue containing non-caseating granulomas, consistent with adrenal sarcoid. Learning points This case highlights an unusual presentation of two uncommon diseases. This case also highlights how separate and potentially unrelated disease processes may occur concomitantly and the importance, therefore, of keeping an open mind when dealing with unusual diagnostic findings. We also hypothesize a potential link between the ACC and sarcoidosis in our patient.

UNEXPECTED INCIDENCE OF LOW BLOOD PRESSURE 2 YEARS AFTER UNILATERAL ADRENALECTOMY FOR PRIMARY ALDOSTERONISM

1989 ◽  
Vol 16 (4) ◽  
pp. 281-286 ◽  
Author(s):  
Richard D. Gordon ◽  
Peter G. Hawkins ◽  
Stephen M. Hamlet ◽  
Terry J. Tunny ◽  
Shelley A. Klemm ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Primary Aldosteronism ◽  
Unilateral Adrenalectomy ◽  
Low Blood Pressure

scholarly journals Aldosterone- and cortisol-cosecreting adrenal adenoma, ovarian hyperthecosis and breast cancer

2020 ◽  
Vol 2020 ◽  
Author(s):  
John J Orrego ◽  
Joseph A Chorny
Keyword(s):  
Breast Cancer ◽  
Estrogen Receptor ◽  
Primary Aldosteronism ◽  
Adrenal Adenoma ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
List Type ◽  
Unilateral Adrenalectomy ◽  
Estrogen Receptor Positive ◽  
Positive Breast Cancer

Summary We describe a 56-year-old postmenopausal woman with hypertension, hypokalemia and severe alopecia who was found to have a 4.5-cm lipid-poor left adrenal mass on CT scan performed to evaluate her chronic right-sided abdominal pain. Hormonal studies revealed unequivocal evidence of primary aldosteronism and subclinical hypercortisolemia of adrenal origin. Although a laparoscopic left adrenalectomy rendered her normotensive, normokalemic and adrenal insufficient for 2.5 years, her alopecia did not improve and she later presented with facial hyperpigmentation acne, worsening hirsutism, clitoromegaly, and an estrogen receptor-positive breast cancer. Further testing demonstrated markedly elevated serum androstenedione and total and free testosterone and persistently undetectable DHEAS levels. As biochemical and radiologic studies ruled out primary adrenal malignancy and obvious ovarian neoplasms, a bilateral salpingo-oophorectomy was undertaken, which revealed bilateral ovarian hyperthecosis. This case highlights how the clinical manifestations associated with hyperaldosteronism and hypercortisolemia masqueraded the hyperandrogenic findings. It was only when her severe alopecia failed to improve after the resolution of hypercortisolism, hyperandrogenic manifestations worsened despite adrenal insufficiency and an estrogen receptor-positive breast cancer was found, did it becomes apparent that her symptoms were due to ovarian hyperthecosis. Learning points: As cortisol cosecretion appears to be highly prevalent in patients with primary aldosteronism, the term ‘Connshing’ syndrome has been suggested. The associated subclinical hypercortisolemia could be the driver for the increased metabolic alterations seen in patients with Conn syndrome. The identification of these dual secretors before adrenal venous sampling could alert the clinician about possible equivocal test results. The identification of these dual secretors before unilateral adrenalectomy could avoid unexpected postoperative adrenal crises. Hyperfunctioning adrenal and ovarian lesions can coexist, and the clinical manifestations associated with hypercortisolemia can masquerade the hyperandrogenic findings.

scholarly journals SAT-LB301 Hyperaldosteronism From Adrenal Adenoma in a Young Patient

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Francisco Javier Lopez Maldonado ◽  
Angel Alfonso Mayorga León ◽  
Alondra Rodriguez Gonzalez ◽  
Eduardo Rafael León Milán ◽  
Carlos Alfonso Morales Chinchillas ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Clinical Symptoms ◽  
Adrenal Adenoma ◽  
Secondary Hypertension ◽  
Primary Hyperaldosteronism ◽  
Blurred Vision ◽  
Plasma Aldosterone Concentration ◽  
Unilateral Adrenalectomy ◽  
Blood Pressure Elevation ◽  
Cardiovascular Morbidity And Mortality

Abstract Introduction Primary aldosteronism (PA) is the most common cause of secondary hypertension (HTN) that is often underdiagnosed. Patients with PA have higher cardiovascular morbidity and mortality than age- and sex- matched patients with essential HTN and the same degree of blood pressure elevation. We present a case of a 17-year old male with primary hyperaldosteronism. Case Report 17-year-old male with no past medical history presented to the emergency department referring bitemporal cephalea, pain in left arm, dyspnea, heart palpitations, a blood pressure (BP) of 220/110 mmHg and a serum potassium of 1.5mmol/L (n. 3.5-5). The patient was admitted to the hospital for better handling of his BP, potassium repositions, monitoring and further management. During hospitalization he referred leg cramps, asthenia, adynamia, nausea, cephalea, and blurred vision. Physical examination demonstrated decreased muscle strength, jugular regurgitation II and hyperreflexia on lower extremities. Thyroid and pulmonary examinations were normal. BP continued elevated despite the concurrent use of 3 antihypertensive drug classes (ARA2, BB, CCB), referred to endocrinologist after being diagnosed with resistant hypertension suspecting of a primary hyperaldosteronism. Lab tests were run obtaining a plasma aldosterone concentration 44ng/dl (n. 4-31), plasma active renin (PRA) 0.05ng/ml/hr (n. 0.2-1.6), aldosterone/PAR ratio 880 (&lt;25 normal). Suprarenal CT scan revealed right suprarenal lesion 2.2 x 2.1 cm, round appearance, hypodense (14 HU) compatible with an adenoma. He underwent unilateral adrenalectomy. Histopathological report from excised adrenal tumor was compatible with a benign adenoma. Patient was discharged with a regularized blood pressure without antihypertensives and normokalemic. No clinical symptoms were reported in follow-up. ConclusionPrimary aldosteronism (PA) is the most common form of secondary hypertension (HTN) with an estimated prevalence of 4% of hypertensive patients in primary care and around 10% of referred patients. PA is characterized by hypertension, hypokalemia, suppressed plasma renin and increased aldosterone excretion. Aldosterone producing adenomas (APAS) account for around 40% and idiopathic hyperaldosteronism for around 60% of PA cases. The most sensitive screening test for PA is the aldosterone-to-renin ratio. After case confirmation, all patients with PA should undergo adrenal CT as the initial study. Unilateral laparoscopic adrenalectomy is the preferential treatment for patients with APAs, and bilateral hyperplasia should be treated with mineralocorticoid antagonist.

scholarly journals Patients With Primary Aldosteronism Respond to Unilateral Adrenalectomy With Long-Term Reduction in Salt Intake

2019 ◽  
Vol 105 (3) ◽  
pp. e484-e493
Author(s):  
Christian Adolf ◽  
Daniel A Heinrich ◽  
Finn Holler ◽  
Benjamin Lechner ◽  
Nina Nirschl ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Primary Aldosteronism ◽  
Sodium Excretion ◽  
Salt Intake ◽  
Urinary Sodium Excretion ◽  
Urinary Sodium ◽  
Care Hospital ◽  
Unilateral Adrenalectomy ◽  
The Impact

Abstract Context High dietary salt intake is known to aggravate arterial hypertension. This effect could be of particular relevance in the setting of primary aldosteronism (PA), which is associated with cardiovascular damage independent of blood pressure levels. The aim of this study was to determine the impact of therapy on salt intake in PA patients. Patients and Methods A total of 148 consecutive PA patients (66 with unilateral and 82 with bilateral PA) from the database of the German Conn’s Registry were included. Salt intake was quantified by 24-hour urinary sodium excretion before and after initiation of PA treatment. Study design Observational longitudinal cohort study. Setting Tertiary care hospital. Results At baseline, unilateral PA patients had a significantly higher urinary sodium excretion than patients with bilateral disease (205 vs 178 mmol/d, P = 0.047). Higher urinary sodium excretion correlated with an increased cardiovascular risk profile including proteinuria, impaired lipid, and glucose metabolism and was associated with higher daily doses of antihypertensive drugs to achieve blood pressure control. In unilateral disease, urinary sodium excretion dropped spontaneously to 176 mmol/d (P = 0.012) 1 year after unilateral adrenalectomy and remained low at 3 years of follow-up (174 mmol/d). In contrast, treatment with mineralocorticoid receptor antagonists (MRA) in bilateral PA patients was not associated with a significant change in urinary sodium excretion at follow-up (179 mmol/d vs 183 mmol/d). Conclusion PA patients consuming a high-salt diet, estimated based on urinary sodium excretion, respond to adrenalectomy with a significant reduction of salt intake, in contrast to MRA treatment.

scholarly journals “Relieving the Pressure” With Unilateral Adrenalectomy in a Case of Chronic Resistant Hypertension From Primary Aldosteronism

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A102-A103
Author(s):  
Danielle Brooks ◽  
Gustavo Fernandez-Ranvier ◽  
Carlos Rios ◽  
Nirali A Shah
Keyword(s):  
Blood Pressure ◽  
Resistant Hypertension ◽  
Primary Aldosteronism ◽  
Complete Resolution ◽  
Surgical Intervention ◽  
Uncontrolled Hypertension ◽  
Laboratory Evaluation ◽  
Plasma Aldosterone Concentration ◽  
Unilateral Adrenalectomy ◽  
Increased Risk

Abstract Introduction: Primary aldosteronism is an under-recognized cause of resistant hypertension that is associated with an increased risk of cardiovascular disease and mortality. While surgical intervention may not lead to complete resolution of hypertension, partial success (defined by reduction in blood pressure and/or medications) can be achieved and future cardiovascular risks can be minimized. We present a case of a patient with primary aldosteronism whose chronic resistant hypertension improved significantly after unilateral adrenalectomy. Clinical Case: A 54-year-old female with resistant hypertension for 17 years and hypokalemia was diagnosed with primary aldosteronism. The patient had uncontrolled hypertension despite atenolol 50 mg, nifedipine XL 60 mg, triamterene-hydrochlorothiazide 37.5-25 mg, lisinopril 40 mg daily. Laboratory evaluation was significant for plasma aldosterone concentration (PAC) 26.8 ng/dL (reference: 0.0–30.0 ng/dL), plasma renin activity (PRA) 0.168 ng/mL/hr (reference: 0.167–5.380 ng/mL/hr), PAC/PRA ratio 159.5, and potassium of 3.2 mmol/L (reference: 3.5–4.5mmol/L). Other workup showed plasma normetanephrines 128 pg/mL (reference: 0–145 pg/mL), metanephrines 25 pg/mL (reference: 0–62 pg/mL), and two normal midnight salivary cortisol tests. Saline infusion testing confirmed primary aldosteronism with a non-suppressed aldosterone level of 15.6 ng/dL (normal &lt;5 ng/dL). Abdominal imaging revealed two low-density right adrenal nodules consistent with adenomas and thickening of the left adrenal gland. The patient underwent adrenal vein sampling (AVS) with cosyntropin stimulation, which showed lateralization to the right adrenal. Despite adding clonidine 0.1 mg three times daily, hydralazine 25 mg three times daily and spironolactone 100 mg daily (which substituted triamterene-hydrochlorothiazide) after AVS, her blood pressure remained uncontrolled with blood pressure ranging from 150–180/90–110 mmHg. A laparoscopic right adrenalectomy was performed. Pathology revealed two adrenal cortical adenomas. At the one-month post-operative visit, her potassium was normal, PAC was 4.3 ng/dL, and blood pressure improved on a reduced regimen of atenolol, lisinopril, and nifedipine. Conclusion: In patients with chronic uncontrolled hypertension due to primary aldosteronism, surgical intervention may not lead to complete resolution of hypertension but may lead to partial clinical success. Residual hypertension may be due to underlying vascular changes associated with long-term exposure to elevated aldosterone. The post-operative PAC in our case was less than 5 ng/dL suggesting biochemical cure and potentially reduced cardiovascular risk. This case emphasizes that adrenalectomy should be considered in resistant hypertension, even if complete clinical cure may not be attainable to reduce future cardiovascular events.

scholarly journals A case report of severe hypoaldosteronism following unilateral adrenalectomy for aldosteronoma

2020 ◽  
Vol 13 (4) ◽  
pp. 192-199
Author(s):  
Lilit Egshatyan
Keyword(s):  
Blood Pressure ◽  
Primary Aldosteronism ◽  
Filtration Rate ◽  
Estimated Glomerular Filtration Rate ◽  
Term Treatment ◽  
Unilateral Adrenalectomy ◽  
Hypertensive Patients ◽  
Long Term Treatment ◽  
Risk Patients

Primary aldosteronism is characterized by hypertension and accounts for about 10% of hypertensive patients. Hyperkalemia and renal disease post adrenalectomy has been described in the literature. We present а case of primary aldosteronism with long standing hypertension (more than 10 years) with severe hypokalemia (1.9 mmol/l). Post unilateral adrenalectomy he had reduction in the blood pressure and became eukalemic. However, after 8 weeks of adrenalectomy patient developed hyperkalemia and increased serum creatinine, which resolved with fludrocortisone and attempt to discontinue fludrocortisone resulted in hyperkalemia and rising creatinine. Screening of developing post-operative hyperkalemia should be actively considered in high-risk patients: older age, longer duration of hypertension, higher levels of aldosterone and severe hypokalemia before surgery, impaired estimated glomerular filtration rate and long-term treatment with spironolactone.

PS 14-84 PREDICTORS OF BLOOD PRESSURE OUTCOME FOLLOWING LAPAROSCOPIC UNILATERAL ADRENALECTOMY IN PRIMARY ALDOSTERONISM

2016 ◽  
Vol 34 (Supplement 1) ◽  
pp. e457
Author(s):  
Kanako Bokuda ◽  
Atsuhiro Ichihara ◽  
Yuki Mizuguchi ◽  
Michita Niiyama ◽  
Yasufumi Seki ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Primary Aldosteronism ◽  
Unilateral Adrenalectomy
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