[Unilateral testicular tumor in adrenogenital syndrome]

In male patients with congenital adrenogenital syndrome (AGS), in addition to the well known adrenal changes, tumor-like testicular proliferations may be observed. They have been known from literature since decades, but have become rare due to modern therapy. Especially in the mild and inapparent cases of AGS, the "tumors" cause clinical and morphological problems. The case of a 34 year old male AGS patient is presented, who underwent resection of a large adrenal cortical adenoma at age 28. Six years later, orchiectomy followed because of a testicular tumor. In frozen sections, the "Leydig cell-like" tumor caused diagnostic difficulties. After usual embedding and examination, the diagnosis of testicular "tumor" in AGS was confirmed. By review of the literature, pathomorphogenesis and morphology of these testicular tumors as well as possible histogenesis of their cells are discussed. Because of the close similarity to Leydig cell tumors, this most important differential diagnosis is especially considered. As a rule, testicular tumors in AGS are no indication for orchiectomy.