Testislerde kitle ile başvuran testiküler ve intraabdominal adrenal rest tümörlü bir konjenital adrenal hiperplazi olgusu

Congenital adrenal hyperplasia (CAH) is a disease that occurs with enzyme defect in steroid synthesis. 21-hydroxylase enzyme deficiency is presented in over 90 per cent of the cases. 21-hydroxylase deficiency has three different clinical forms which are salt wasted, simple virilizing and non-classical. Because of the enzyme deficiency, steroid synthesis decreases and adrenocorticotropic hormon release increases. All adrenal cortical cells increase. % 8.2 of the male patients with CAH have testicular mass. It is presented in patients with no treatment and generally bilateral. Because of the morphological and functional similarity with adrenal tissue, it is called testicular adrenal rest tumor (TART). A 20 year-old man presented with bilateral testicular masses. His history is accorded with puberty precocity. Laboratory tests showed hypoglycemia without symptoms. Further laboratory and imaging tests showed primary adrenal insufficiency, bilateral adrenal hyperplasia, intraabdominal and testicular masses. After steroid replacement therapy, bilateral orchiectomy and unilateral adrenal mass excition performed.