OBJECTIVE: There are a few studies regarding the prevalence of testicular adrenal rest tumors (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 2-18 years of age, were included in the study. Fifty-five patients had 21-hydroxylase deficiency (21-OHD) and 5 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; 8 patients had 21-OHD, and 3 had 11β-OHD. The youngest TART patient was 4 years old, whereas 8 patients RTs were at puberty. Only 2 patients had tight metabolic control: 8 patients had stage 2, 1 had stage 4, and 2 had stage 5 rest tumors. In 4 patients with stage 2 TARTs, tumors disappeared after high-dose steroid treatment and did not recur. Shrinkage of tumor was observed in 2 patients. Testis-sparing surgery was performed in 1 patient with stage 5 tumor. Gonadal functions were normal in patients with partially regressed tumors. Two patients became fathers of healthy male off-springs.
CONCLUSIONS: Detection and treatment of 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.
Written by:
Aycan Z, Bas VN, Cetinkaya S, Agladioglu SY, Tiryaki T. Are you the author?
Clinics of Pediatric Endocrinology, School of Medicine, Yıldırım Beyazıt University, and Clinics of Pediatric Endocrinology, Dr. Sami Ulus Research and Training Hospital of Women's and Children's Health and Diseases, Ankara, Turkey.
Reference: Clin Endocrinol (Oxf). 2012 Oct 12. Epub ahead of print.
doi: 10.1111/cen.12033
PubMed Abstract
PMID: 23057653
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