577V 49 16 2012 Testicular adrenal rest tumors TART are consequences of delayed diagnosis andorundertreatment in patients with congenital adrenal hyperplasia CAH We describe a case ofCAH with T ID: 942204
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577V 49 16, 2012 CCCCCAAAAASSSSSEEEEE R R R REEEEPPPPP Testicular adrenal rest tumors (TART) are consequences of delayed diagnosis and/orundertreatment in patients with congenital adrenal hyperplasia (CAH). We describe a case ofCAH with TART who presented with bilateral macroorchidism. He was managed with Congenital adrenal hyperplasia, Macroorchidism, Testicular adrenal rest tumors.Correspondence to: 578V 49 16, 2012EPORTSTesticular biopsy showed diffuse sheets and lobules oflarge, polygonal cells with abundant eosinophiliccytoplasm. The cells had ill defined borders separated byLeydig cells were also seen. However, no germ cells,appreciated. There was no evidence of any infiltrativedisorder. Based on these features a diagnosis of TART wasramipril 5 mg daily.We describe a patient of CAH with testicular adrenal resttumor based on isosexual precocious puberty, shortorchidomegaly and elevated 17 OH-progesterone. Thepossibility of 11 genital ambiguity. The increase in 17OH-progesterone inpatients with 11 this enzyme converts 11-deoxy cortisol to cortisol, whilebiosynthetic pathway.TART have not been demonstrated in non classical-hydroxylase or 11 CAH [6]. This can be possibly be explained because ofin 11 -hydroxylase deficiency, 11 deoxy cortisol hascortisol like activity, thereby some degree ofmaintained resulting in modest rise in ACTH. Thepossible reason for having TART in the present case maybe
due to longstanding, untreated CAH. There areas some authors have described a steroid gradientas some authors have described a steroid gradientConsequences of TART include discomfort due tomassive orchidomegaly and infertility. Infertility is. Infertility isischemia and fibrosis and suppression of gonadotropinsdue to weaker adrenal androgens, consequently resultingin atrophy of the tubules [7,8]. The present case hadTART results in regression of testicular size and results in regression of testicular size andtherapy led to decrease in the testicular size after a followup visit at 6 months; however, spermatogenesis was not1.Claahsen-van der Grinten HL, Otten BJ, Sweep FC, SpanPN, Ross HA, Meuleman EJ, 2.Avila NA, Premkumar A, Merke DP. Testicular adrenal3.Claahsen-van der Grinten HL, Sweep FCGJ, Blickman JG,rest tumors in male children with congenital adrenal4.Souverijns G, Peene P, Keuleers H, Vanbockrijck M.5.Reisch N, Scherr M, Flade L, Bidlingmaier M, Schwarz6.Claahsen-van der Grinten HL, Otten BJ, Stickelbroeckrest tumours in congenital adrenal hyperplasia. Best Pract7.Claahsen-van der Grinten HL, Otten BJ, Hermus AR,Sweep FC, Hulsbergen-van de Kaa CA. Testicular adrenal8.Bercovici JP, Fiet J, Gibault L,Volant A, Abalain JH, Flochstudies). J Steroid Biochem Mol Biols. 2005;93:67-72.9.White CP, Carter JN. Adrenal and testicular tumors andazoospermia in congenital adrenal hyperplasia. Aust Ne