Medizinische Klinik und Poliklinik IV, Klinikum der Universität, Ludwig-Maximilians-Universität München, Munich, Germany.
Research Unit Analytical Pathology and.
JCI Insight. 2019 Sep 5;4(17):130356. doi: 10.1172/jci.insight.130356.
Recent genetic examinations and multisteroid profiles have provided the basis for subclassification of aldosterone-producing adenomas (APAs). The objective of the current study was to produce a comprehensive, high-resolution mass spectrometry imaging (MSI) map of APAs in relation to morphometry, immunohistochemical profiles, mutational status, and clinical outcome. The study cohort comprised 136 patients with unilateral primary aldosteronism. Matrix-assisted laser desorption/ionization-Fourier transform-ion cyclotron resonance MSI was conducted, and metabolite profiles were analyzed with genotype/phenotype information, including digital image analysis from morphometry and IHC of steroidogenic enzymes. Distinct molecular signatures between KCNJ5- and CACNA1D-mutated APAs with significant differences of 137 metabolites, including metabolites of purine metabolism and steroidogenesis, were observed. Intratumor concentration of 18-oxocortisol and 18-hydroxycortisol were inversely correlated with the staining intensity of CYP11B1. Lower staining intensity of CYP11B1 and higher levels of 18-oxocortisol were associated with a higher probability of complete clinical success after surgery. The present study demonstrates distinct metabolomic profiles of APAs in relation to tumor genotype. In addition, we reveal an inverse correlation between cortisol derivatives and CYP11B1 and the impact of 18-oxocortisol and CYP11B1 on clinical outcome, which provides unprecedented insights into the pathophysiology, clinical features, and steroidogenesis of APAs.
最近的基因检测和多甾体谱分析为醛固酮瘤(APA)的亚分类提供了依据。本研究的目的是生成 APA 的全面、高分辨率的质谱成像(MSI)图谱,以关联形态计量学、免疫组织化学特征、突变状态和临床结果。研究队列包括 136 例单侧原发性醛固酮增多症患者。进行了基质辅助激光解吸/电离-傅里叶变换离子回旋共振 MSI,并用基因型/表型信息分析代谢物谱,包括来自形态计量学和类固醇生成酶的 IHC 的数字图像分析。在 KCNJ5-和 CACNA1D 突变的 APA 之间观察到明显的分子特征,有 137 种代谢物存在显著差异,包括嘌呤代谢物和类固醇生成代谢物。18-氧皮质醇和 18-羟基皮质醇的肿瘤内浓度与 CYP11B1 的染色强度呈负相关。CYP11B1 的染色强度较低和 18-氧皮质醇水平较高与手术后完全临床成功的可能性更高相关。本研究表明 APA 的代谢组学图谱与肿瘤基因型有关。此外,我们揭示了皮质醇衍生物与 CYP11B1 之间的反比关系,以及 18-氧皮质醇和 CYP11B1 对临床结果的影响,这为 APA 的病理生理学、临床特征和类固醇生成提供了前所未有的见解。