Testicular Adrenal Rest Tumors Associated With Congenital Adrenal Hyperplasia

Abstract

Congenital adrenal hyperplasia is an autosomal recessive disorder that impairs the production of two essential hormones, cortisol and aldosterone. This deficiency results in the overproduction of adrenocorticotropic hormone and overgrowth of the adrenal tissue. Specific forms of congenital adrenal hyperplasia can result in ambiguous genitalia, precocious puberty, short stature, and testicular adrenal rest tumors. Common sonographic findings of testicular rest tissue are multifocal hypoechoic lesions and a spokelike vascularity with multiple peripheral vessels. The sonographer needs to understand the correlation between congenital adrenal hyperplasia and testicular adrenal rest tumors so the testicular masses are not misdiagnosed.

Keywords

congenital adrenal hyperplasia adrenal rest tumors cortisol aldosterone adrenocorticotropic hormone

References

DeGroot LJ , Jameson JL : Endocrinology: Defects of Adrenal Steroidogenesis. 5th ed. New York, Elsevier Saunders, 2006.

Avila NA , Premkumar A. , Shawker TH , Jones JV , Laue L. , Cutler GB : Testicular adrenal rest tissue in congenital adrenal hyperplasia: findings at gray-scale and color Doppler US. Radiology 1996;198:99—104.

Marieb EN : Human Anatomy & Physiology. 6th ed. San Francisco, Pearson Benjamin Cummings, 2003.

Avila NA , Shawker TS , Jones JV , Cutler GB , Merke DP : Testicular adrenal rest tissue in congenital adrenal hyperplasia: serial sonographic and clinical findings. AJR Am J Roentgenol 1999;172:1235—1238.

Saada J. , Grebille AG , Aubry MC , Rafii A. , Dumez Y. , Benachi A. : Sonography in prenatal diagnosis of congenital adrenal hyperplasia . Prenat Diagn 2004;24:627—630.

Moshang TJ , Bell LM : Pediatric Endocrinology: The Requisites in Pediatrics. St. Louis, MO, Elsevier Mosby , 2005.

Stikkelbroeck NMML , Suliman HM , Otten BJ , Hermus AR , Blickman JGB , Jagber BJ : Testicular adrenal rest tumor in postpubertal males with congenital adrenal hyperplasia: sonographic and MR features. Eur Radiol 2003;13:1597—1603.

Rumack CM , Wilson SR , Charboneau JW Diagnostic Ultrasound. 3rd ed. St. Louis, MO, Mosby, 2005.

Nagbamine WH , Mehta SV , Vade A. : Testicular adrenal rest tumors in a patient with congenital adrenal hyperplasia: sonographic and magnetic resonance imaging findings. J Ultrasound Med 2005;24:1717—1720.