Adrenocorticotropin-independent bilateral macronodular adrenocortical hyperplasia: Immunohistochemical studies of steroidogenic enzymes and post-operative course in two men

Norio Wada, Mitsumasa Kubo, Hiromichi Kijima, Tatsuya Ishizuka, Tomoko Saeki, Takao Koike, Hironobu Sasano

Research output: Contribution to journalArticlepeer-review

32 Citations (Scopus)

Abstract

We treated two men with Cushing's syndrome due to adrenocorticotropin (ACTH)-independent bilateral macronodular adrenocortical hyperplasia (AIMAH). In both patients, plasma ACTH was low and plasma cortisol was not suppressed by a high dose of dexamethasone (8 mg) but was remarkably responsive to exogenous ACTH. The adrenal glands were extremely enlarged and contained multiple nodules composed of large clear cells and small compact cells. The immunoreactivity of P-450(17α) was predominant in the small compact cells, while that of 3β-hydroxysteroid dehydrogenase (3 β-HSD) was observed exclusively in the large clear cells. Among various adrenocortical disorders, differential expression of 3 β-HSD and P-450(17α) in clear and compact cells has heretofore been demonstrated only in AIMAH. Total adrenalectomy was done for one patient, and partial adrenalectomy for the other. In the former patient, the normal diurnal rhythm of plasma ACTH was restored 11 months postoperatively. In the latter patient, the normal dynamics in the hypothalamic-pituitary-adrenal axis became evident 15 months after surgery. Thus AIMAH is apparently a primary adrenocortical disorder and is not due to abnormalities of the hypothalamus or pituitary.

Original languageEnglish
Pages (from-to)583-587
Number of pages5
JournalEuropean Journal of Endocrinology
Volume134
Issue number5
DOIs
Publication statusPublished - 1996 May

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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