Prevalence and long-term follow-up outcomes of testicular adrenal rest tumours in children and adolescent males with congenital adrenal hyperplasia.

Abstract

OBJECTIVE:

There are a few studies regarding the prevalence of testicular adrenal rest tumours (TARTs) in boys and adolescent males with congenital adrenal hyperplasia (CAH), and there is little information regarding the treatment outcomes in patients with TARTs. The aim of this study was to determine the long-term treatment outcomes in boys and adolescent males with CAH.

PATIENTS AND METHODS:

Sixty boys and adolescent males with CAH, who were between 2 and 18 years of age, were included in the study. Fifty-five patients had 21-hydroxylase deficiency (21-OHD), and five patients had 11-β hydroxylase deficiency (11β-OHD). All patients were screened for TARTs by scrotal ultrasonography (US) performed by an experienced radiologist.

RESULTS:

TART prevalence was 18·3% in 2-18 years' of age; eight patients had 21-OHD, and three had 11β-OHD. The youngest patient with TART was 4 years old, whereas eight patients with RTs were at puberty. Only two patients had tight metabolic control: eight patients had stage 2, one had stage 4, and two had stage five rest tumours. In four patients with stage 2 TARTs, tumours disappeared after high-dose steroid treatment and did not recur. Shrinkage of tumour was observed in two patients. Testis-sparing surgery was performed in one patient with stage five tumour. Gonadal functions were normal in patients with partially regressed tumours. Two patients became fathers of healthy male off-springs.

CONCLUSIONS:

Detection and treatment for TARTs in children with CAH at younger ages, earlier stages, may prevent infertility in adulthood. Therefore, we recommend that scrotal US screening should be performed in every 1-2 years starting from early childhood.

• Authors: Zehra Aycan, Veysel N Bas, Semra Cetinkaya, Sebahat Yilmaz Agladioglu, Tuğrul Tiryaki
• Journal: Clinical endocrinology (Clin Endocrinol (Oxf)) Vol. 78 Issue 5 Pg. 667-72 (May 2013) ISSN: 1365-2265 [Electronic] England
• PMID: 23057653 (Publication Type: Journal Article)
• Copyright: © 2012 Blackwell Publishing Ltd.

Topics

• Adolescent
• Adrenal Hyperplasia, Congenital (epidemiology)
• Adrenal Rest Tumor (epidemiology)
• Child
• Humans
• Male