SUN-398 A Case of Bilateral Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia Due to 21 Hydroxylase Deficiency

Abstract

Case study: This is a 28-year-old male, who was diagnosed since childhood with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. He is a product of consanguineous marriage and one of his sister is suffering from the same problem. He was maintained on hydrocortisone tablets twice a day “10 mg and 15 mg”, in addition to fludrocortisone 0.1 mg once daily. His current problem stared since two years. He was not compliant to his medicine, because of depression. Then, he noticed darkening of his skin. Additionally, he was suffering from fatigue and erectile dysfunction as well as primary infertility. Clinically, he was hyperpigmented, and there was a testicular mass felt in each testis. He had an MRI Abdomen which showed the left adrenal gland contained a large lobulated mass filled with macroscopic fat measuring 3.7*2.9 cm and the right adrenal gland had another fat rich lobulated mass of 1.6*1.1 cm. Ultrasound testes revealed bilateral irregular hypoechoic lesion close to the rete testis. The right testicular measured 2.6*0.7 cm and the left one measured 2.6*0.5 cm. Pertaining laboratory results showed ACTH 26.4 pmol/L (1.6 - 13.9), 17-hydroxyprogesterone 855 nmol/L ( 1.8-10.3), the repeated result was 974 nmol/L , FSH <0.1 , and total testosterone 2.75 nmol/L ( 8.64 - 29). The patient was started on night dose of long acting steroid dexamethasone 0.5 mg once daily, in addition to fludrocortisone 0.1 mg once daily. He was evaluated regularly over two years. The skin hyper pigmentation has got better. His sexual function and sperm count improve with time, but he is still seeking for fertility. The repeated laboratory results showed ACTH dropped to 1.5 pmol/L (1.6 - 13.9), 17-hydroxyprogesterone 0.66 nmol/L (1.8-10.3).The latest MRI adrenals had shown reduction in the size of both adrenal masses to less than 1 cm. The ultrasound testes showed the left mass has reduced in size from 2.6*0.5 cm to 1.4*0.3 cm and the right testicular adrenal rest tumor has resolved completely. Discussion: Congenital adrenal hyperplasia is a rare inherited disease. Inappropriate management might result in rare complications such as testicular adrenal rest tumor. The use of a long acting glucocorticoid to suppress ACTH might result in regression of testicular adrenal rest tumor or complete. In conclusion: Testicular adrenal rest tumor is a benign condition which might occurs in patient with congenital adrenal hyperplasia. The tumor might regress or even disappear with proper ACTH suppression.