Gomez-Sanchez Celso E, Gomez-Sanchez Elise P
aEndocrine Section, G.V. (Sonny) Montgomery, VA Medical CenterbDepartment of MedicinecDepartment of Pharmacology and Toxicology, University of Mississippi Medical Center, Jackson, Mississippi, USA.
Curr Opin Endocrinol Diabetes Obes. 2016 Jun;23(3):242-8. doi: 10.1097/MED.0000000000000248.
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.
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.
APAs have a complex structure and expression of steroidogenic enzymes.
原发性醛固酮增多症是高血压的主要病因；醛固酮瘤（APA）导致约一半的原发性醛固酮增多症；其余病例则由肾上腺球状带细胞特发性增生引起。最近在大量APA的离子通道和泵中发现了令人惊讶的各种突变。本综述探讨了APA及其周围肾上腺的组织学和分子学方面。
分别针对CYP11B2和CYP11B1酶（醛固酮和皮质醇合成中的最后一种酶）的特异性抗体，首次使得对APA和相邻肾上腺内细胞的类固醇生成能力进行研究成为可能。表达CYP11B2的细胞可能分散和/或成簇分布于整个正常肾上腺球状带。APA中表达CYP11B2的细胞数量差异很大；一些根本不表达，但被表达CYP11B2的细胞包围，有些细胞成簇或形成微结节。一些APA还包含同时表达CYP11B1和CYP17A1的细胞。在一些样本中，对APA相邻组织的分析检测到了此前仅与APA相关的离子通道和泵突变。
APA具有复杂的结构和类固醇生成酶的表达。
