Asymptomatic testicular adrenal rest tumours in adolescent and adult males with congenital adrenal hyperplasia: basal and follow-up investigation after 2.6 years.
OBJECTIVE
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.


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 > or =30% decrease in the sum of the longest diameter(s) of the lesion(s), was found in six patients; tumour increase, defined as > or =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 prolonged ACTH suppression, had resulted in better adrenal suppression in only one patient.


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.
Stikkelbroeck
Nike M M L
NM
Department of Paediatric Endocrinology, University Medical Centre Nijmegen, Nijmegen, The Netherlands.
Hermus
Ad R M M
AR
Suliman
Harold M
HM
Jager
Gerrit J
GJ
Otten
Barto J
BJ
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications* / Q000188:drug therapy*; D000314:Adrenal Rest Tumor / Q000097:blood / Q000000981:diagnostic imaging / Q000209:etiology*; D000328:Adult; D000735:Androstenedione / Q000037:antagonists & inhibitors; D003907:Dexamethasone / Q000008:administration & dosage* / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D005640:Follicle Stimulating Hormone / Q000097:blood; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage* / Q000627:therapeutic use; D006801:Humans; D006854:Hydrocortisone / Q000008:administration & dosage* / Q000627:therapeutic use; D007986:Luteinizing Hormone / Q000097:blood; D008297:Male; D012463:Saliva / Q000378:metabolism; D013736:Testicular Neoplasms / Q000097:blood / Q000000981:diagnostic imaging / Q000209:etiology*; D013739:Testosterone / Q000097:blood; D014463:Ultrasonography
D005938:Glucocorticoids; D013739:Testosterone; D000735:Androstenedione; D003907:Dexamethasone; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D006854:Hydrocortisone