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

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.