Evidence for endogenous LH suppression in a man with bilateral testicular tumors and congenital adrenal hyperplasia.

The need for a functional classification of “Leydig cell” tumors in men with congenital adrenal hyperplasia (CAH) is exemplified by the present case of a 19 year old boy with this disorder, who developed bilateral testicular masses despite replacement with hydrocortisone. During basal conditions, in which he received hydrocortisone, 25 mg/day, urinary 17-ketosteroids and pregnanetriol were 33 and 18.5 mg/24 h, respectively. Concomitantly, mean plasma 17-hydroxy-progesterone (17OHP) concentrations, 32.2 (A.M.), 58.8 (P.M.), ng/ml, were increased 30–50-fold, while mean plasma cortisol (F), 7.7 (A.M.), 12.3 (P.M.) μg/dl, testosterone (T), 485 (A.M.) 493 (P.M.) ng/dl and estradiol (E2) 12.4 (A.M.), 16.3 (P.M.) pg/ml, concentrations were within normal limits. Episodic fluctuations of plasma T, E2 and 170HP were demonstrated in both morning and afternoon samples, with pulses occurring at approximately 90 min intervals. By contrast plasma LH levels, pulsatile in the morning, fell in the afternoon with no evident...