Testicular Adrenal Rest Tumor in Congenital Adrenal Hyperplasia Patients Attending Assuit University Children Hospital

Congenital adrenal hyperplasia (CAH) is one of the most frequent endocrine disorders. It is characterized by deficient activity of one of the enzymes necessary for adrenal Cortisol synthesis. Autosomal recessive inheritance of deficient 21-hydroxylase (21 OH) activity accounts for more than 90% of cases. [1]

One of the most important and frequently detected complications in males with congenital adrenal hyperplasia (CAH) is the development of testicular adrenal rest tumours (TARTs). . [2],[3]

Testicular adrenal rest tumor is a benign tumor originating from adrenal cells within the testicles; the adrenal cells migrate at 8 weeks of gestation from the urogenital ridge along with the gonadal cells to the testicle. The exact mechanism underlying tumor growth and development is still not well understood . [4],[5]

The correct diagnosis of TART is important for differential diagnosis with malignant testis tumors, which need orchiectomy, as opposed to TART, usually treated exclusively with exogenous steroids. [6].

Although TARTs are always benign, their location in the rete testis could obstructs the seminiferous tubules and causes gonadal dysfunction and infertility. Therefore, it is important to detect and treat TARTs at an early stage, especially in adolescents or young adults. [5],[7]

testicular ultrasound should be done in order to evaluate the presence of these lesions, its size, location, and characteristics. [9]

TARTs in children have mostly been presented as case reports in the literature, and only a limited number of studies have described the prevalence of TARTs in large populations of children . [2],[8]

As not all male CAH patients are affected by this complication, attempts have been made to identify the risk factors for the development of TARTs . [7]

Inclusion criteria

• All male patients diagnosed as congenital adrenal hyperplasia based on clinical manifestations and laboratory investigations from age of 4 years to age of 18 years.

Exclusion criteria

• Patients with other adrenal insufficiency cause and Congenital adrenal hyperplasia that progressed to true precocious puberty