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Adrenogenital Syndrome, more properly defined as Congenital Adrenal Hyperplasia (CAH), is related to the enzyme 21-
beta-hydroxylase deficiency, with impaired glucocorticoids and aldosterone syntheses and increased ACTH synthesis.
This report describes a case of a monorchid patient suffering from Adrenogenital Syndrome and Leydig cell tumor of his
testis. A right orchidectomy with implantation of testis prosthesis was performed, after informing the patient on the consequences
of his castration and obtaining his consent. Histology showed a testis measuring 4x3x2.5 cm with a 6 cm long
spermatic cord; there was a yellowish, well-defined nodule measuring 3.5×1.5 cm, surrounded by normal parenchyma.
This nodule had morphologic and immunohistochemical characteristics of a Leydig cell tumor, even found in the spermatic
cord; those cells showed positivity to inibine, MART-1 and vimentine.
