Department of Clinical and Molecular Endocrinology, Tokyo Medical and Dental University Graduate School, Tokyo 113-8519, Japan.
Endocr J. 2011;58(7):543-51. doi: 10.1507/endocrj.k10e-402. Epub 2011 Apr 27.
Primary aldosteronism (PA), an autonomous aldosterone hypersecretion from adrenal adenoma and/or hyperplasia, and subclinical Cushing syndrome (SCS), a mild but autonomous cortisol hypersecretion from adrenal adenoma without signs or symptoms of Cuhing's syndrome, are now well-recognized clinical entities of adrenal incidentaloma. However, the clinicopathological features of PA associated with SCS (PA/SCS) remain unknown. The present study was undertaken to study the prevalence of PA/SCS among PA patients diagnosed at our institute, and characterize their clinicopathlogical features. The prevalence of PA/SCS was 8 of 38 PA patients (21%) studied. These 8 PA/SCS patients were significantly older and had larger tumor, higher serum potassium levels, lower basal plasma levels of aldosterone, ACTH and DHEA-S as well as lower response of aldosterone after ACTH stimulation than those in 12 patients with aldosterone-producing adenoma without hypercortisolism. All 8 PA/SCS patients showed unilateral uptake by adrenal scintigraphy at the ipsilateral side, whereas the laterality of aldosterone hypersecretion as determined by adrenal venous sampling varied from ipsilateral (3), contralateral (2), and bilateral side (2). 6 PA/SCS patinets who underwent adrenalectomy required hydrocortisone replacement postoperatively. Histopathological analysis of the resected adrenal tumors from 5 PA/SCS patients revealed a single adenoma in 3, and double adenomas in 2, with varying degrees of positive immunoreactivities for steroidgenic enzymes (3β-HSD, P450(C17)) by immunohistochemical study as well as CYP11B2 mRNA expression as measured by real-time RT-PCR. In conclusion, PA/SCS consists of a variety of adrenal pathologies so that therapeutic approach differs depending on the disease subtype.
原发性醛固酮增多症（PA）是由肾上腺腺瘤和/或增生引起的醛固酮自主性分泌过多，亚临床库欣综合征（SCS）是由肾上腺腺瘤引起的轻度但自主性皮质醇分泌过多，没有库欣综合征的迹象或症状，现在是公认的肾上腺意外瘤的临床实体。然而，PA 伴 SCS（PA/SCS）的临床病理特征尚不清楚。本研究旨在研究我院诊断的 PA 患者中 PA/SCS 的患病率，并描述其临床病理特征。PA/SCS 的患病率为 38 例 PA 患者中的 8 例（21%）。这 8 例 PA/SCS 患者明显年龄较大，肿瘤较大，血清钾水平较高，基础血浆醛固酮、ACTH 和 DHEA-S 水平较低，ACTH 刺激后醛固酮反应较低，而 12 例无高皮质醇血症的醛固酮瘤患者则较低。所有 8 例 PA/SCS 患者在同侧肾上腺闪烁扫描中均显示单侧摄取，而通过肾上腺静脉取样确定的醛固酮分泌异常的侧位性从同侧（3）、对侧（2）和双侧（2）不等。6 例接受肾上腺切除术的 PA/SCS 患者术后需要氢化可的松替代治疗。对 5 例 PA/SCS 患者切除的肾上腺肿瘤进行组织病理学分析，发现 3 例为单个腺瘤，2 例为双腺瘤，免疫组织化学研究显示类固醇生成酶（3β-HSD、P450(C17))的阳性免疫反应程度不同，以及通过实时 RT-PCR 测量的 CYP11B2 mRNA 表达。总之，PA/SCS 包含多种肾上腺病变，因此治疗方法因疾病亚型而异。
