IMPE Abstracts

Introduction: Vast majority of children and adolescents with adrenocortical tumor (ACT) present clinical signs of androgen excess. Long-term exposure to sex steroids can activate the GnRH pulse generator and trigger central precocious puberty, as shown in treated 21-hydroxilase deficiency, McCune-Albright syndrome and testotoxicosis. ACTs are rare in pediatric patients and, therefore, the knowledge about characteristics of pubertal outcome after tumor resection. We present data of a cohort treated in a single center of Southern Brazil, where pediatric ACT incidence is 10-15 times higher than worldwide.

Methods: Retrospective study of pubertal characteristics of patients treated for ACT during the period of 1966-2021.

Results: Thirty-eight patients were females; median chronological age (CA) at diagnosis was 2.7 years (0.01-13.4) and bone age (BA) 5.5 years (0.75-13.5). Virilizing syndrome with or without hypercortisolism was present in 87% of the cases; tumor stages I and II represented 66% of the cohort; stage III, 28% and stage, IV 6%. Puberty started 7 years after tumor resection in girls and 7.5 years in boys. The median CA at telarche was 9.3 years (8.3-11.3) and mean CA at menarche 11.75±1.2 years. In boys, median CA at gonadarche was 9.9 years (9.2-13.6). Central precocious puberty (CPP) occurred in nine patients (7 girls). One female patient presented pseudo-precocious puberty while on mitotane chemotherapy. Median interval between tumor resection and start of CPP was 0.4 years. CA > 4 years independently influenced CPP occurrence [OR 9.3 (2-45); P<0.01] in multivariate analysis. Besides no influence in logistic regression, BA-SDS was inversely correlated with age of puberty onset: -0.52 (P<0.01) for girls and -0.81 (P<0.01) for boys.

Conclusion: CPP may be part of the natural history of ACT in children, especially in those diagnosed at older ages. Close follow up after tumor removal is mandatory not only for surveillance of tumor recurrence but also for the possibility of precocious puberty.