Testicular histopathology in congenital lipoid adrenal hyperplasia: a light and electron microscopic study.

This paper describes the testicular histopathology in 2 male patients with congenital lipoid adrenal hyperplasia who underwent orchiectomy at age 7 (case 1) and 12 (case 2) years, respectively. In both cases, seminiferous tubules were normal in diameter (mean: case 1 88.7 microm; case 2 115.9 microm) and consisted of an age-appropriate number of type A spermatogonia (mean: case 1 1.7/tubule; case 2 4.0/tubule) and an increased number of Sertoli cells (mean: case 1 34.7/tubule; case 2 27.9/tubule). The interstitial space was abundant and contained normal to increased numbers of Leydig cells (mean: case 1 4.0/ tubule; case 2 6.1/tubule) that were filled with lipid droplets. We suspect that lipid accumulation in Leydig cells is consistent with defective conversion of cholesterol into pregnenolone and thus constitutes the characteristic feature of this disease, while the remaining findings appear to be common to disorders associated with hypergonadotropic hypogonadism.