Approach to testicular adrenal rest tumors in children and adolescents with congenital adrenal hyperplasia

Sirmen Kızılcan Çetin, Z. Aycan
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Abstract

Testicular adrenal rest tumor (TART) is a long-standing, significant complication of congenital adrenal hyperplasia (CAH). Although it is the main reason for infertility in adult patients with CAH, little is known about the development mechanism, genotype-phenotype features, and exact treatment options of TART. However, in the literature, there are mainly case reports and only a few studies in the pediatric period on this subject. The critical point is the early detection of tumors to prevent infertility in adulthood. This paper reviews the embryological, clinical, diagnostic, genetic features, and treatment alternative of TART and discusses the early screening options.
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先天性肾上腺增生儿童及青少年睾丸肾上腺休息肿瘤的探讨
睾丸肾上腺休息肿瘤(TART)是先天性肾上腺增生(CAH)的一个长期的、显著的并发症。尽管它是成年CAH患者不孕的主要原因,但人们对TART的发生机制、基因型-表型特征以及确切的治疗方案知之甚少。然而,在文献中,这一主题主要是病例报告,只有少数研究在儿科时期。关键是肿瘤的早期发现,以防止成年后不孕。本文综述了TART的胚胎学、临床、诊断、遗传特征和治疗选择,并讨论了早期筛查的选择。
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