Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia.
OBJECTIVE
Early diagnosis and treatment of testicular adrenal rest tumors (TART) is important for gonadal functions and fertility protection in boys with congenital adrenal hyperplasia (CAH). In this descriptive study, we investigated the prevalence of TART in boys with 21-hydroxylase deficient (21OHD) CAH followed in our pediatric endocrine clinic.


METHODS
The study group consisted of 14 male patients with a mean age of 9.6 ± 5.1 (range: 0.8-18.3) years. Six (42.9%) of the 14 patients were diagnosed as having salt-wasting type (SW) and eight (57.1%) patients - as having the simple virilizing (SV) form of 21OHD. Mean age at diagnosis was 2.9 ± 2.7 (range: 0.03-6.3) years. Two different radiologists performed scrotal ultrasonography. Chronological age, bone age, and anthropometric measurements were evaluated. Serum adrenocorticotropic hormone (ACTH), 17-alpha-hydroxyprogesterone (17OHP) and androstenedione levels were also evaluated in all patients during the follow-up period.


RESULTS
Scrotal ultrasonography revealed bilateral TART in two patients (14.3%) and testicular microlithiasis (TM) in four patients (28.6%). One patient had both TART and TM bilaterally. During the follow-up period, the mean serum adrenocorticotropic hormone, 17OHP and androstenedione levels in the total group of patients were 130.0 ± 179.1 pg/mL (21.7-726.5), 5.8 ± 3.3 ng/mL (0.8-11.4) and 4.3 ± 4.1 (0.2-11.0) ng/mL, respectively.


CONCLUSIONS
Microlithiasis or TART may be frequently encountered during the follow-up of patients with CAH. In order to prevent late complications including infertility, we suggest that ultrasonographic evaluations be performed yearly in all male CAH patients.
Uludağ University Faculty of Medicine, Department of Pediatric Endocrinology, Bursa, Turkey. edpapatya@yahoo.com
Esra Deniz Papatya
ED
Çakir
Fatma Sentürk
FS
Mutlu
Erdal
E
Eren
Aliye Ozlem
AO
Paşa
Halil
H
Sağlam
Omer
O
Tarim
D019326:17-alpha-Hydroxyprogesterone / Q000097:blood; D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D000314:Adrenal Rest Tumor / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000365:Age Determination by Skeleton; D000735:Androstenedione / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D012611:Scrotum / Q000000981:diagnostic imaging; D013255:Steroid 21-Hydroxylase / Q000378:metabolism; D013736:Testicular Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D016896:Treatment Outcome; D014463:Ultrasonography / Q000379:methods
D000735:Androstenedione; D019326:17-alpha-Hydroxyprogesterone; D000324:Adrenocorticotropic Hormone; D013255:Steroid 21-Hydroxylase