Background: In pediatric patients with adrenocortical tumors (ACT), morbidity and mortality rates have been extensively evaluated. However, there are almost no data on the late consequences of early exposure to high androgen levels on pubertal development and on final height (FH) in these patients.
Objective: To investigate the impact of early exposure to androgen excess on gonadotropin-dependent pubertal development and on final height (FH) of patients with childhood ACT.
Design: Retrospective cohort study of 63 pediatric patients (69.8% female) with virilizing ACT followed in a single institution from September 1975 until September 2017.
Methods: Data between patients with normal puberty (n=26) and pubertal disorders central precocious puberty (CPP, n=7) and early fast puberty (EFP, n=3) were compared.
Results: At diagnosis of ACT, median age was 25.8 months and duration of signs, 6 months; stature SDS was 0.5 (−3.6 3.9) and bone age advancement was 14.7 months (−27.9 85.4). To date, 58.7% of the patients developed gonadotropin-dependent puberty: 26 had normal puberty, 7 CPP, and 3 EFP. GnRH analogues effectively treated CPP/EFP. Tall stature and older age at diagnosis of ACT were associated with risk of CPP [RR 4.17 (95% CI 1.17 14.80)] and pubertal disorders [RR 3.0 (95% CI 1.04 8.65)], respectively. Recurrence/metastasis were associated with CPP [RR 4.17 (95% CI 1.17 14.80)] and pubertal disorders [RR 3.0 (95% CI 1.12 8.02)]. Among the 19 patients that reached FH, stature SDS dropped from 1.4 to −0.02 since diagnosis of ACT (P=0.01). Seventeen of them achieved normal FH. There was no difference in FH SDS between patients with normal puberty and pubertal disorders (P=0.75).
Conclusions: This study clearly shows that gonadotropin-dependent pubertal disorders are more common than previously expected in patients with childhood virilizing ACTs. Additionally, it confirms that FH is usually not impaired, reiterating the good prognosis for FH in these patients. Our study reinforces the importance of close and prolonged endocrinology follow-up after surgery, not only to detect ACT-related complications, but also to promptly identify and treat consequences of early exposure to androgen excess.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology