IMPE Abstracts

Testicular adrenal rest tumors (TARTs) are one of the most frequent comorbidities in Congenital Adrenal Hyperplasia (CAH) males resulting in infertility and hypergonadotropic hypogonadism; however, different prevalence rates have been reported and the predisposing factors are not known. Objective: To assess the TARTs prevalence in a pediatric CAH cohort, followed in a same center since diagnosis, and to identify the predictor factors. Patients and methods: data of 29 CAH patients (14 salt-wasters-SW, 8 simple virilizing-SV and 7 nonclassical-NC) were retrospectively evaluated. Laboratorial analysis included ACTH, 17OHP, androstenedione, testosterone (T), FSH, LH, and renin levels, which were analyzed in the last 5 yrs and testicular ultrasound. All patients had CAH molecular diagnosis and data of patients with/without TARTs were compared using Mann Whitney and Student’s t tests. Results: TARTs were observed in 31% of patients (5/14 SW, 4/8 SV and none of NC patients); all patients, except one, presented bilateral tumors. Mean age at TART diagnosis was 13.5 ± 4 yrs. The youngest patient was 6 yrs old, the SV diagnosis was performed at 5 yrs with bone age of 11 yrs. Mean ACTH levels, in the last 5 yrs, were higher in patients with TART than those without (ACTH 476 ± 379 vs. 169 ± 104 pg/mL, P<0.05). There was no difference in renin, androstenedione, testosterone or 17-hydroxiprogesterone levels between patients with and without TART. None patient developed hypergonadotropic hypogonadism until last evaluation. Mean glucocorticoid (in hidrocortisone equivalent doses) and fludrocortisone doses (of last five years) in patients with TARTs was 13.2 3.8 mg/m2/day and 75 mg/day, respectively. Conclusion: in our series, a high frequency of TARTs was observed in classical CAH patients, especially during puberty. We suggest that chronic stimulatory effect of ACTH, instead of severity of clinical form or renin levels, seems to be the major role for TARTs development. These data suggest the TARTS screening since childhood.