Based on histopathology, steroid-producing properties, and ACTH responsiveness, these testicular tumors are believed to consist of adrenal tissue. Aberrant adrenal tissue has been identified in 7.5% of normal testes by Dahl et al. (44). In the embryological phase, the adrenal glands develop in the immediate vicinity of the gonads. Aberrant adrenal tissue may descend with the testis or ovary along the course of their supplying arteries and end up in the testes, celiac plexus, broad ligament, or ovary (44-48). The aberrant adrenal cells are believed to retain their potential for glucocorticoid production and to respond with hyperplasia to increased ACTH stimulation. The testicular tumors in males with CAH are, therefore, often called adrenal rest tumors.
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