Large bilateral adrenal incidentalomas complicating untreated 11 beta hydroxylase deficiency in the third decade of life - A case report

The occurrence of bilateral giant adrenal incidentalomas is reported in a 2 2-year-old man who was evaluated for urinary tract infection. The right gla nd measured 16 cm, the left one 6 cm at computed tomography. Height was 145 cm, blood pressure 190/100 mm Hg. Testes were not palpable. Laboratory investigations revealed elevated levels of 17 hydroxyprogesteron e : > 50 ng/ml (n < 1,1) ; 11 desoxycortisol : 919 nmol/l (n < 30) : testos terone : 19 ng/ml (n < 0.7) and ACTH : 1 402 ng/l (n < 48). Karyotype was 4 6 XX. The patient was a female pseudohermaphrodite with congenital adrenal 11 bet a hydroxylase deficiency. Adrenal masses responded to glucocorticoid therapy with marked reduction of their size after six months. We confirm previous recommendations that patients with adrenal incidentalom a should be checked for congenital adrenal hyperplasia.