A Case of Congenital Adrenal Hyperplasia Combined with a Testicular Adrenal Rest Tumor and Adrenal Incidentaloma.

Gyu Rang Cho; Hee Won Chueh; Jung Pyo Kim; Jin A Jung; Jae Ho Yoo; Sung Kook Yoon; Kyu Geun Hwang

doi:10.3803/jkes.2007.22.5.365

Case Report A Case of Congenital Adrenal Hyperplasia Combined with a Testicular Adrenal Rest Tumor and Adrenal Incidentaloma.: Gyu Rang Cho, Hee Won Chueh, Jung Pyo Kim, Jin A Jung, Jae Ho Yoo, Sung Kook Yoon, Kyu Geun Hwang; Endocrinology and Metabolism 2007;22(5):365-370
DOI: https://doi.org/10.3803/jkes.2007.22.5.365
Published online: October 1, 2007

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¹Department of Pediatrics, Dong-A University College of Medicine.
²Department of Diagnostic Radiology, Dong-A University College of Medicine.

Abstract

The fundamental defect among patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylse deficiency is the inability to synthesize cortisol and aldosterone adequately. Ineffective cortisol synthesis signals the hypothalamus and pituitary to increase the production of corticotropin releasing hormone and adrenocorticotropic hormone, respectively. Consequently, the adrenal glands become hyperplastic. It is well known that an adrenal adenoma can develop from hyperplastic tissue under increased corticotropin stimulation of the adrenal cortex in patients that are suffering with CAH. The etiologic mechanism of adrenal incidentaloma remains uncertain, but several hypotheses have been suggested. A testicular adrenal rest tumor has been reported to form in association with the excessive secretion of adrenal androgen by inadequate control after adolescence in CAH. We present a case of poorly controlled salt-losing CAH due to 21-hydroxylase deficiency combined with a testicular adrenal rest tumor and adrenal incidentaloma.

Keywords: Adrenal incidentaloma;Congenital adrenal hyperplasia;Testicular adrenal rest tumor;

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