Horm Metab Res 2015; 47(13): 967-972
DOI: 10.1055/s-0035-1565225
Review

Steroid Hormone Production in Patients with Aldosterone Producing Adenomas

M. Moors
1   Department of Internal Medicine, Radboud University Medical Center, Nijmegen, The Netherlands
,
T. A. Williams
2   Division of Internal Medicine and Hypertension, Department of Medical Sciences, University of Turin, Turin, Italy
3   Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximilians-Universität München, Munich, Germany
,
J. Deinum
1   Department of Internal Medicine, Radboud University Medical Center, Nijmegen, The Netherlands
,
G. Eisenhofer
4   Institute of Clinical Chemistry and Laboratory Medicine, Technische Universität Dresden, Dresden, Germany
5   Department of Internal Medicine III, Technische Universität Dresden, Dresden, Germany
,
M. Reincke
3   Medizinische Klinik und Poliklinik IV, Klinikum der Ludwig-Maximilians-Universität München, Munich, Germany
,
J. W. M. Lenders
1   Department of Internal Medicine, Radboud University Medical Center, Nijmegen, The Netherlands
5   Department of Internal Medicine III, Technische Universität Dresden, Dresden, Germany
› Author Affiliations

Abstract

Primary aldosteronism encompasses 2 major underlying causes: (1) aldosterone producing adenoma and (2) bilateral adrenal hyperplasia. In addition to the aldosterone excess, increased production of other compounds of the steroidogenic pathways may be involved. Until recently, most studies examined the production of steroids other than aldosterone in tumor tissue, urine, or peripheral plasma samples, but several new studies have also addressed steroid levels in adrenal venous blood samples using liquid chromatography tandem mass spectrometry. Plasma and tissue levels of several precursors of aldosterone with mineralocorticoid activity are higher in patients with aldosterone producing adenomas than in those with bilateral hyperplasia. These include corticosterone, deoxycorticosterone, and their 18-hydroxylated metabolites. Similarly, urinary, peripheral, and adrenal venous concentrations of the hybrid steroids 18-oxocortisol and 18-hydroxycortisol are higher in patients with aldosterone producing adenomas than in bilateral hyperplasia. Differences in the pathophysiology and in clinical and biochemical phenotypes caused by aldosterone producing adenomas and bilateral adrenal hyperplasia may be related to the differential expression of steroidogenic enzymes, and associated to specific underlying somatic mutations. Correct appreciation of differences in steroid profiling between aldosterone producing adenomas and bilateral adrenal hyperplasia may not only contribute to a better understanding of the pathogenesis of primary aldosteronism but may also be helpful for future subtyping of primary aldosteronism.



Publication History

Received: 20 August 2015

Accepted: 03 November 2015

Article published online:
14 December 2015

Georg Thieme Verlag
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
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