| Print Date: | May 20, 2013, 2:48 am |
| Title | Adrenal Rest |
| Text | Congenital adrenal hyperplasia is one of the most frequently encountered autosomal recessive genetic defects. The carrier state is present in nearly 1 on 60 persons, with Hispanics and Ashkenazi Jews being the most common carriers. The disorder is caused by an enzyme defect, usually 21-hydroxylase, however 11-hydroxylase and 17-hydroxylase defects are also seen. This leads to impaired cortisol production, and dysfunction of the pituitary/hypothalamic axis. The result is hyperandrogenism, hypoadrenalism, and elevated ACTH. Ectopic adrenal tissue, called adrenal rests, are a common finding in this population. Depending on the source, some 20-50% of persons have adrenal rests, most commonly found in the testes, celiac plexus, and broad ligaments. In a recent study, 24% of male patients with CAH had testicular adrenal rests. Ultrasound is very sensitive to the detection of adrenal rests, which usually appear as a hypoechoic intratesticular mass, with varying vascularity. Testicular dysfunction is frequently associated with these benign masses. Treatment with high dose glucocorticoids usually results in reduction in the size of the mass and improvement in testicular function. Resistant masses are treated with conservative testicular-sparing procedures. |
| References: | Middleton W, Kurtz A, Hertzberg,B, Ultrasound: The Requesites. St. Louis: Mosby, 2004
Stikkelbroeck, Otten. High prevalence of testicular adrenal rest tumors, impaired spermatogenesis, and leydig cell failure in adolescent and adult males with congenital adrenal hyperplasia. J Clin Endo & Metab 2001;86:5721-8 Avila N.A., Premkumar A. Testicular adrenal rest tissue in congenital adrenal hyperplasia: findings at gray-scal and color doppler US. Radiology 1996;198:99-104 |
| Contributor | Zachary M Alexander (National Naval Medical Center Bethesda) |
| Peer Reviewer | Ellen M Chung (Uniformed Services University) |
| Record Number | : 6501 |
| Created | 2005-05-01 21:18:19-04 |
| Modified | 2005-07-31 14:33:11.201934-04 |
| Category: | Endocrine |
| Location: | Genitourinary |
| Sublocation: | None Selected |
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