Celso E Gomez-Sanchez1, Elise P Gomez-Sanchez. 1. aEndocrine Section, G.V. (Sonny) Montgomery, VA Medical CenterbDepartment of MedicinecDepartment of Pharmacology and Toxicology, University of Mississippi Medical Center, Jackson, Mississippi, USA.
Abstract
PURPOSE OF REVIEW: Primary aldosteronism is a major cause of hypertension; aldosterone-producing adenomas (APA) cause about half of primary aldosteronism; idiopathic hyperplasia of adrenal glomerulosa cells are responsible for the rest. A surprising variety of mutations have recently been identified in ion channels and pumps in a significant number of APA. The present review addresses histological and molecular aspects of APA and the surrounding adrenal. RECENT FINDINGS: Specific antibodies against the CYP11B2 and CYP11B1 enzymes, the last enzyme in aldosterone and cortisol synthesis, respectively, allow for the first time study of the steroidogenic capabilities of cells within the APA and adjacent adrenal. Cells expressing CYP11B2 may be scattered and/or in clusters throughout the normal adrenal zona glomerulosa. APA differ widely in the number of cells expressing CYP11B2; some did not express it at all, but were surrounded by cells, some in clusters or micronodules, that expressed CYP11B2. Some APAs also comprised cells expressing both CYP11B1 and CYP17A1. In some samples, analysis of the tissue adjacent to APA detected ion channel and pump mutations heretofore associated only with APA. SUMMARY: APAs have a complex structure and expression of steroidogenic enzymes.
PURPOSE OF REVIEW: Primary aldosteronism is a major cause of hypertension; aldosterone-producing adenomas (APA) cause about half of primary aldosteronism; idiopathic hyperplasia of adrenal glomerulosa cells are responsible for the rest. A surprising variety of mutations have recently been identified in ion channels and pumps in a significant number of APA. The present review addresses histological and molecular aspects of APA and the surrounding adrenal. RECENT FINDINGS: Specific antibodies against the CYP11B2 and CYP11B1 enzymes, the last enzyme in aldosterone and cortisol synthesis, respectively, allow for the first time study of the steroidogenic capabilities of cells within the APA and adjacent adrenal. Cells expressing CYP11B2 may be scattered and/or in clusters throughout the normal adrenal zona glomerulosa. APA differ widely in the number of cells expressing CYP11B2; some did not express it at all, but were surrounded by cells, some in clusters or micronodules, that expressed CYP11B2. Some APAs also comprised cells expressing both CYP11B1 and CYP17A1. In some samples, analysis of the tissue adjacent to APA detected ion channel and pump mutations heretofore associated only with APA. SUMMARY: APAs have a complex structure and expression of steroidogenic enzymes.
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