A Rare Case of Adrenocorticotropic Hormone (ACTH)-independent Macroadrenal Hyperplasia Showing Ectopic Production of ACTH

Minoru Iwata; Yutaka Oki; Teruyo Okazawa; Shin Ishizawa; Chihiro Taka; Katsuya Yamazaki; Kazuyuki Tobe; Junya Fukuoka; Hironobu Sasano; Tetsuo Nishikawa

doi:10.2169/internalmedicine.51.7547

CASE REPORTS

A Rare Case of Adrenocorticotropic Hormone (ACTH)-independent Macroadrenal Hyperplasia Showing Ectopic Production of ACTH

Minoru Iwata, Yutaka Oki, Teruyo Okazawa, Shin Ishizawa, Chihiro Taka, Katsuya Yamazaki, Kazuyuki Tobe, Junya Fukuoka, Hironobu Sasano, Tetsuo Nishikawa

Author information

Minoru Iwata
The First Department of Internal Medicine, Faculty of Medicine, University of Toyama, Japan
Community Medical Support Unit, Faculty of Medicine, University of Toyama, Japan
Yutaka Oki
Department of Medicine, Hamamatsu University School of Medicine, Japan
Teruyo Okazawa
The First Department of Internal Medicine, Faculty of Medicine, University of Toyama, Japan
Shin Ishizawa
Department of Pathology, Faculty of Medicine, University of Toyama, Japan
Department of Pathology, Toyama Prefectural Central Hospital, Japan
Chihiro Taka
The First Department of Internal Medicine, Faculty of Medicine, University of Toyama, Japan
Katsuya Yamazaki
Kawai Clinic, Japan
Kazuyuki Tobe
The First Department of Internal Medicine, Faculty of Medicine, University of Toyama, Japan
Junya Fukuoka
Department of Surgical Pathology, Toyama University Hospital, Japan
Hironobu Sasano
Department of Pathology, Tohoku University School of Medicine, Japan
Tetsuo Nishikawa
Division of Endocrinology and Metabolism, Endocrinology and Diabetes Center, Department of Medicine, Yokohama Rosai Hospital, Japan

Keywords: ACTH-independent macroadrenal hyperplasia, ectopic ACTH production, autocrine/paracrine regulatory mechanism

JOURNAL OPEN ACCESS

2012 Volume 51 Issue 16 Pages 2181-2187

DOI https://doi.org/10.2169/internalmedicine.51.7547

Details

Abstract

A 57-year-old Japanese man presented with drug-resistant hypertension without Cushingoid features. Endocrinological tests revealed autonomous secretion of cortisol with suppressed plasma adrenocorticotropic hormone (ACTH). Imaging examinations showed multiple macronodules in the bilateral adrenal gland. These findings were consistent with subclinical Cushing's syndrome caused by ACTH-independent macronodular adrenal hyperplasia (AIMAH). Left adrenalectomy was performed and the resected adrenal lesion was consistent with the pathological diagnosis of AIMAH. Furthermore, in resected tissue, we demonstrated intraadrenal production of ACTH by immunohistochemical analysis and RIA. This is a very rare case of AIMAH showing ectopic production of ACTH, which may be associated with autonomous cortisol secretion.

Corresponding author

Register with J-STAGE for free!