Adrenal rest tumors may interfere directly (mechanically or paracrine) or indirectly (endocrine) with testicular function. The mechanical effect of the tumors can be considerable because of their central location adjacent to the mediastinum testis. It can lead to obstruction of the vascular supply and compression and atrophy of the seminiferous tubules. Pathological examination revealed that in cases of large tumors, the residual testicular parenchyma was abnormal and showed atrophy, sclerosis, or immaturity and decreased or absent spermatogenesis (28). However, around small nodules, the testicular parenchyma was generally normal (28). Murphy et al. showed in one patient that germ cells close to the mediastinum and the tumor were reduced and sper-matogenesis was impaired. However, testicular aspiration in other areas showed normal spermatogenesis (53).
In addition to direct mechanical effects (obstruction or destruction), tumors may have a paracrine effect on the surrounding tissue, i.e., steroids produced by the tumors could be toxic to Sertoli cells or germ cells (53).
Indirect (endocrine) effects of testicular tumors in CAH on fertility could result from steroid secretion by the tumor, with suppression of the hypothalamic-pituitary-gonadal axis. The association between infertility, testicular adrenal rest tumors, and depressed serum LH and FSH levels in males with CAH has been described (5,6,39,43,54). However, the effect of steroid secretion by the tumor cannot be readily separated from the effect of excess steroids secreted by the adrenal glands.
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