Asymptomatic Testicular Adrenal Rest Tumours in Adolescent and Adult Males with Congenital Adrenal Hyperplasia: Basal and Follow-up Investigation After 2.6 Years

Abstract

Aim:To study the course of asymptomatic testicular adrenal rest tumours in patients with congenital adrenal hyperplasia (CAH) and the association between tumour changes and glucocorticoid therapy adjustments. Patients and Methods: Fifteen male patients with CAH (21-hydroxylase deficiency), in whom asymptomatic testicular adrenal rest tumours had been found at a baseline investigation, underwent scrotal ultrasonography and venous blood sampling (for LH, FSH and testosterone) on average 2.6 years later. The level of hormonal control was assessed by measurement of androstenedione in three diurnal saliva samples. Data on changes in glucocorticoid therapy since baseline were obtained from the patients' records. Results: Tumour decrease, defined as >30% decrease in the sum of the longest diameter(s) of the lesion(s), was found in six patients; tumour increase, defined as >20% increase, in six and stable tumours in three patients. All three patients with overtreatment showed tumour decrease and of the six patients with undertreatment only one showed tumour decrease. Tumour increase was not only observed in undertreated patients but also in patients with adequate treatment. Changing the night dose of hydrocortisone into dexamethasone, to obtain Conclusions: Tumour decrease could be achieved by aiming at adrenal oversuppression, but the required high glucocorticoid doses may induce side effects. In asymptomatic tumours in young male patients with CAH, a practical guideline could be to optimise adrenal suppression to a maximal tolerable glucocorticoid dose and to offer analysis and cryopreservation of semen as soon as the patient can be motivated.

Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

“…ACTH may be an important stimulator of tumour growth, because ACTH receptors are present on the tumour tissue, and some authors describe a relationship between poor hormonal control and progression of tumour growth (22,23). Although adequacy of substitution therapy was not systematically assessed in our patients, none of our patients had documented periods of poor hormonal control in early childhood or puberty.…”

Restoration of fertility by gonadotropin replacement in a man with hypogonadotropic azoospermia and testicular adrenal rest tumors due to untreated simple virilizing congenital adrenal hyperplasia

“…However, if high gonadotropin levels are found, they can be indicative of a testicular failure (22,30). Whereas some investigators found that TARTs regressed and spermatogenesis was reactivated when glucocorticoid therapy was instituted or intensified (11,31), others report that patients remain azoospermic (27,32). The use of dexamethasone (0.25-0.5 mg p.o.…”

Testicular Tumors in Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency Show Functional Features of Adrenocortical Tissue

“…However, intensifying of glucocorticoid treatment with suppression of ACTH secretion is not always successful in reducing tumor size and even in well-controlled CAH patients, with normal or suppressed plasma ACTH levels, testicular adrenal rest tumors are found (2,3,12). Therefore, most probably other unknown factors contribute to tumor growth.…”