促肾上腺皮质激素非依赖性双侧肾上腺皮质大结节性增生：一例报告及免疫组织化学研究

Adrenocorticotropic hormone-independent bilateral adrenocortical macronodular hyperplasia: a case report and immunohistochemical studies.

作者信息

Koizumi S, Beniko M, Ikota A, Mizumoto H, Matsuya K, Matsuda A, Sakuma S, Mashio Y, Kunita H, Okamoto K

机构信息

Department of Internal Medicine, Kin-ikyo Chuo Hospital, Sapporo, Japan.

出版信息

Endocr J. 1994 Aug;41(4):429-35. doi: 10.1507/endocrj.41.429.

DOI:10.1507/endocrj.41.429

PMID:8528359

Abstract

A 55-year-old woman developed Cushing's syndrome due to ACTH-independent bilateral adrenocortical macronodular hyperplasia. Plasma ACTH was undetectable, and was not stimulated by administration of metyrapone, CRH, or insulin. Hypercortisolism was not suppressed by a high dose of dexamethasone, but was responsive to ACTH. Both adrenal glands were enlarged with a total weight of 200 g, and contained multiple nodules composed of two cell types (large clear cells and small compact cells). In immunohistochemical studies, P450c17 immunoreactivity was predominantly observed in small compact cortical cells, while that of 3 beta HSD was observed exclusively in large clear cortical cells. This pattern of expression of steroidogenic enzymes as well as histological and clinical features is considered to be unique to ACTH-independent bilateral adrenocortical macronodular hyperplasia.

摘要

一名55岁女性因促肾上腺皮质激素（ACTH）非依赖性双侧肾上腺皮质大结节增生而患上库欣综合征。血浆ACTH检测不到，且服用甲吡酮、促肾上腺皮质激素释放激素（CRH）或胰岛素后均未受到刺激。高剂量地塞米松不能抑制高皮质醇血症，但对ACTH有反应。双侧肾上腺均增大，总重量为200克，包含由两种细胞类型（大透明细胞和小致密细胞）组成的多个结节。在免疫组织化学研究中，P450c17免疫反应主要在小致密皮质细胞中观察到，而3β-羟类固醇脱氢酶（3βHSD）的免疫反应仅在大透明皮质细胞中观察到。这种类固醇生成酶的表达模式以及组织学和临床特征被认为是ACTH非依赖性双侧肾上腺皮质大结节增生所特有的。