Background: Infertility is a serious complication among male congenital adrenal hyperplasia (CAH) patients which is often caused by testicular adrenal rest tumors (TART). TART are already present in childhood and early infancy in CAH patients. The incidence of TART in neonates without CAH has not yet been described in detail before. Objective: To study the prevalence of testicular adrenal rests in non-CAH neonates. Design: Descriptive study. Setting: Radboud University Nijmegen Medical Centre, The Netherlands. Patients and Methods: 115 testis samples of 89 male infants without CAH who died within the neonatal period were histologically examined. Main Outcome Measures: Prevalence of adrenal rest tissue in the neonatal testes. Results: Adrenal rests were found in 4 samples (3.5%). These adrenal nodules were all located within the epididymis; only in 1 sample a nodule was found close to the rete testis but still within the caput of the epididymis. No nodules were found within the testes. Of the 4 children with adrenal rests, 3 had urological malformations. Conclusion: The incidence of testicular adrenal rests in non-CAH neonates is low. Further studies are necessary to study the incidence of TART in CAH infants and detect typical risk factors in this patient group.

Keywords:
Testicular adrenal rest tumors, Congenital adrenal hyperplasia
1.
White PC, Speiser PW: Congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Endocr Rev 2000;21:245–291.
2.
Claahsen-van der Grinten HL, Otten BJ, Hermus AR, Sweep FC, Hulsbergen-van de Kaa CA: Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia can cause severe testicular damage. Fertil Steril 2008;89:597–601.
3.
Stikkelbroeck NM, Otten BJ, Pasic A: High prevalence of testicular adrenal rest tumors, impaired spermatogenesis and Leydig cell failure in adolescent and adult males with congenital adrenal hyperplasia. J Clin Endocrinol Metab 2001;86:5721–5728.
4.
Jääskeläinen J, Kiekara O, Hippeläinen M, Voutilainen R: Pituitary gonadal axis and child rate in males with classical 21-hydroxylase deficiency. J Endocrinol Invest 2000;23:23–27.
5.
Cabrera MS, Vogiatzi MG, New MI: Long-term outcome in adult males with classic congenital adrenal hyperplasia. J Clin Endocrinol Metab 2001;86:3070–3078.
6.
Reisch N, Flade L, Scherr M, Rottenkolber M, Pedrosa Gil F, Bidlingmaier M, Wolff H, Schwarz HP, Quinkler M, Beuschlein F, Reincke M: High prevalence of reduced fecundity in men with congenital adrenal hyperplasia. J Clin Endocrinol Metab 2009;94:1665–1670.
7.
Avila NA, Premkumar A, Shawker TH, Jones JV, Laue L, Cutler GB Jr: Testicular adrenal rest tissue in congenital adrenal hyperplasia: findings at Gray-scale and color Doppler US. Radiology 1996;198:99–104.
8.
Vanzulli A, DelMaschio A, Paesano P, Braggion F, Livieri C, Angeli E, Tomasi G, Gatti C, Severi F, Chiumello G: Testicular masses in association with adrenogenital syndrome: US findings. Radiology 1992;183:425–429.
9.
Claahsen-van der Grinten HL, Sweep FC, Blickman JG, Hermus AR, Otten BJ: Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Eur J Endocrinol 2007;157:339–344.
10.
Shanklin DR, Richardson AP Jr, Rothstein G: Testicular hilar nodules in adrenogenital syndrome. Am J Dis Child 1963;106:243–250.
11.
Hatano O, Takakusu A, Nomura M, Morohashi K: Identical origin of adrenal cortex and gonad revealed by expression profiles of Ad4BP/SF-1. Genes Cells 1996;1:663–671.
12.
Rutgers JL, Young RH, Scully RE: The testicular ‘tumor’ of the adrenogenital syndrome. A report of six cases and review of the literature on testicular masses in patients with adrenocortical disorders. Am J Surg Pathol 1988;12:503–513.
13.
Sullivan JG, Gohel M, Kinder RB: Ectopic adrenocortical tissue found at groin exploration in children: incidence in relation to diagnosis, age and sex. BJU Int 2005;95:407–410.
14.
Dahl EV, Bahn RC: Aberrant adrenal cortical tissue near the testis in human infants. Am J Pathol 1962;40:587–598.
15.
Val P, Jeays-Ward K, Swain A: Identification of a novel population of adrenal-like cells in the mammalian testis. Dev Biol 2006;299:250–256.
16.
Claahsen-van der Grinten HL, Otten BJ, Sweep FC, Span PN, Ross HA, Meuleman EJ, Hermus AR: Testicular tumors in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency show functional features of adrenocortical tissue. J Clin Endocrinol Metab 2007;92:3674–3680.
17.
Hu L, Monteiro A, Johnston H, King P, O’Shaughnessy PJ: Expression of Cyp21a1 and Cyp11b1 in the fetal mouse testis. Reproduction 2007;134:585–591.
18.
Luo X, Ikeda Y, Parker KL: A cell-specific nuclear receptor is essential for adrenal and gonadal development and sexual differentiation. Cell 1994;77:481–490.
19.
Zubair M, Oka S, Parker KL: Transgenic expression of Ad4BP/SF-1 in fetal adrenal progenitor-1 cells leads to ectopic adrenal formation. Mol Endocrinol 2009;23:1657–1667.
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2010
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