Abstract
Introduction: Most children with adrenocortical tumors (ACTs) present with accelerated growth and skeletal maturation at diagnosis, which potentially compromises their adult heights (AHs). Knowledge about growth and pubertal patterns after ACT resection is scarce. This study presents the pubertal and auxological development of patients treated for ACT and followed up at a single pediatric endocrinology service in Brazil. Methods: Retrospective cohort study including 63 patients (47 girls) followed up between 1966 and 2021. Pubertal and auxological data from ACT diagnosis to AH were analyzed. Results: At diagnosis, the patients had median values of bone age (BA) more advanced than chronological age (CA), height standard deviation score greater than target height (TH-SDS), and predicted adult height (PAH-SDS) lower than TH-SDS. The difference between BA and CA decreased gradually during follow-up and the PAH-SDS moved closer to the TH-SDS 7 years after tumor resection. Puberty started at a median CA of 9.3 (8.3–11.3) years in girls and 9.9 (9.2–13.6) years in boys. Nine patients (6 girls) developed central precocious puberty (CPP), which was influenced by a CA >4 years at diagnosis. The difference between AH-SDS and TH-SDS was not significant (p = 0.3). The factors independently associated with AH below TH were CA >4 years at diagnosis, time between clinical manifestation and diagnosis >1 year, and development of CPP. Conclusion: Most patients treated for ACT during childhood attained AH within the TH despite presenting with advanced skeletal maturation at diagnosis. Development of CPP was not infrequent.
Plain Language Summary
Most children with adrenocortical tumors (ACTs) present androgens excess at diagnosis, which cause virilization and acceleration of growth and skeletal maturation. In this manner, the children have tall stature at diagnosis but a potential comprise of their adult heights (AHs). This study reviewed pubertal and growth data from ACT diagnosis to AH of 63 patients followed up in a single pediatric endocrinology service in Brazil in the last 50 years. At diagnosis, the patients of this study had height greater than that would be expected considering their parents heights (target height), but with an estimate to be shorter than their parents as adults, based on their advanced skeletal maturation. During the follow-up, the skeletal maturation gradually became adequate to the chronological age and the majority of the patients attained AH adequate for their target height (TH). Older age at diagnosis (>4 years), longer time between clinical manifestation and diagnosis, and precocious puberty were factors that impacted in AH below target height. Nine of 51 patients evaluated developed precocious puberty, which was not an infrequent finding.