Adrenocorticotropic Hormone-Independent Bilateral Adrenocortical Macronodular Hyperplasia: A Case Report and Immunohistochemical Studies

Shigeki Koizumi, Matsuo Beniko, Akemi Ikota, Hiroaki Mizumoto, Kumiko Matsuya, Akira Matsuda, Satoshi Sakuma, Yasuo Mashio, Haruhiko Kunit, Kenzo Okamoto, Hironobu Sasano

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11 Citations (Scopus)

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, P450cl7 immunoreactivity was predominantly observed in small compact cortical cells, while that of 3βHSD 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.

Original languageEnglish
Pages (from-to)429-435
Number of pages7
Journalendocrine journal
Volume41
Issue number4
DOIs
Publication statusPublished - 1994

Keywords

  • ACTH-independent bilateral adrenocortical macronodular hyperplasia
  • Cushing's syndrome
  • Steroidogenic enzymes

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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