Diagnosis and Treatment > Medication > Eplerenone

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Yasufumi Seki Departments of Endocrinology and Hypertension, Tokyo, Japan

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Satoshi Morimoto Departments of Endocrinology and Hypertension, Tokyo, Japan

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Naohiro Yoshida Departments of Endocrinology and Hypertension, Tokyo, Japan

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Kanako Bokuda Departments of Endocrinology and Hypertension, Tokyo, Japan

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Nobukazu Sasaki Departments of Endocrinology and Hypertension, Tokyo, Japan

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Midori Yatabe Departments of Endocrinology and Hypertension, Tokyo, Japan

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Junichi Yatabe Departments of Endocrinology and Hypertension, Tokyo, Japan

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Daisuke Watanabe Departments of Endocrinology and Hypertension, Tokyo, Japan

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Satoru Morita Departments of Diagnostic Imaging and Nuclear Medicine, Tokyo, Japan

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Keisuke Hata Departments of Urology, Kidney Center, Tokyo, Japan

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Tomoko Yamamoto Departments of Surgical Pathology, Tokyo Women’s Medical University, Tokyo, Japan

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Yoji Nagashima Departments of Surgical Pathology, Tokyo Women’s Medical University, Tokyo, Japan

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Atsuhiro Ichihara Departments of Endocrinology and Hypertension, Tokyo, Japan

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Summary

Primary aldosteronism (PA) is more common than expected. Aberrant adrenal expression of luteinizing hormone (LH) receptor in patients with PA has been reported; however, its physiological role on the development of PA is still unknown. Herein, we report two unique cases of PA in patients with untreated Klinefelter’s syndrome, characterized as increased serum LH, suggesting a possible contribution of the syndrome to PA development. Case 1 was a 39-year-old man with obesity and hypertension since his 20s. His plasma aldosterone concentration (PAC) and renin activity (PRA) were 220 pg/mL and 0.4 ng/mL/h, respectively. He was diagnosed as having bilateral PA by confirmatory tests and adrenal venous sampling (AVS). Klinefelter’s syndrome was suspected as he showed gynecomastia and small testes, and it was confirmed on the basis of a low serum total testosterone level (57.3 ng/dL), high serum LH level (50.9 mIU/mL), and chromosome analysis. Case 2 was a 28-year-old man who had untreated Klinefelter’s syndrome diagnosed in his childhood and a 2-year history of hypertension and hypokalemia. PAC and PRA were 247 pg/mL and 0.3 ng/mL/h, respectively. He was diagnosed as having a 10 mm-sized aldosterone-producing adenoma (APA) by AVS. In the APA, immunohistochemical analysis showed co-expression of LH receptor and CYP11B2. Our cases of untreated Klinefelter’s syndrome complicated with PA suggest that increased serum LH levels and adipose tissues, caused by primary hypogonadism, could contribute to PA development. The possible complication of PA in hypertensive patients with Klinefelter’s syndrome should be carefully considered.

Learning points:

  • The pathogenesis of primary aldosteronism is still unclear.

  • Expression of luteinizing hormone receptor has been reported in aldosterone-producing adenoma.

  • Serum luteinizing hormone, which is increased in patients with Klinefelter’s syndrome, might contribute to the development of primary aldosteronism.

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S Hussain Department of Endocrinology, St Bartholomew's Hospital, London, UK

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E Panteliou Department of Endocrinology, St Bartholomew's Hospital, London, UK

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D M Berney Department of Pathology, St Bartholomew's Hospital, London, UK

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R Carpenter Department of Surgery, St Bartholomew's Hospital, London, UK

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M Matson Department of Radiology, St Bartholomew's Hospital, London, UK

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A Sahdev Department of Radiology, St Bartholomew's Hospital, London, UK

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M Bell Department of Endocrinology, Galway University Hospital, Galway, UK

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E O'Sullivan Department of Endocrinology, Galway University Hospital, Galway, UK

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W M Drake Department of Endocrinology, St Bartholomew's Hospital, London, UK

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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.

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