Intensified glucocorticoid-mineralocorticoid therapy is the preferred treatment for testicular tumors in CAH. By increasing the glucocorticoid dose, ACTH secretion is suppressed and the adrenal rest tissue is no longer stimulated. The need for tumor shrinkage and the side effects of increasing glucocorticoid therapy must be carefully balanced, especially in asymptomatic cases (55). In 1988, Rutgers et al. reported tumor shrinkage in 75% of 16 patients after increasing the glucocorticoid dose (28). In 1997, Walker et al. reviewed 75 cases of testicular tumors in CAH and stated that the majority of the masses regressed with an increase in glucocorticoid dose (26). Increasing the glucocorticoid dose can also lead to improved semen quality.
If tumor size does not decrease with increasing glucocorticoid therapy, surgical intervention may be considered. Walker et al. reviewed the surgical management of these tumors and showed that the predominant intervention was orchiectomy in 19 of 28 patients (68%) (26). In these patients, the tumors were steroid resistant or (initially) misdiagnosed as LCTs. Walker performed a testis-sparing enucleation in three boys with CAH with steroid-unresponsive testicular tumors. Postoperative MRI and ultrasound of the testis in two of the three patients showed good vascular flow in the remaining tissue and no evidence of recurrent tumor. No semen analysis could be performed.
Because fertility prognosis in CAH males is uncertain, cryopreservation of the semen can be proposed to young male patients. In the case of unwanted infertility, assisted reproduction can be considered. When there is obstructive azoospermia, testic-ular aspiration and intracytoplasmatic sperm injection may offer a solution, as reported by Murphy et al. (53).
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