IMPE Abstracts

The estimated global prevalence of pediatric adrenocortical tumors (PACTs) is 0.5 cases/million population. However, malignant adrenocortical carcinomas is rare and accounts for 0.2% of all childhood malignancies, with considerable variability in incidence rates per different geographical areas. The south/southeast regions of Brazil are known to have a high incidence of PACTs because of the founder effect associated with a germline pathogenic variant of tumor suppressor gene T...

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 resectio...

Context: Premature adrenarche in girls is defined biochemically by an increase in adrenal androgen (DHEA and DHEAS) levels above the age-specific reference range before age 8 years. Recently, increased levels of 11-oxyandrogens have also been observed in girls with premature adrenarche. On the other hand, epigenetic control of some genes that code for enzymes that participate in the synthetic pathway to 11-oxyandrogens (e.g., CYP11B1) has been describ...

The adrenal cortex undergoes constant cellular turnover: capsular stem cells give rise to cortical progenitor and differentiated steroidogenic cells of the zona glomerulosa (zG). Despite knowledge of their existence, little is known about these progenitor populations and have not previously been identified in single cell RNA sequencing (scRNA-Seq) transcriptomic analysis. We postulated that additional ACTH stimulation in the context of 21-hydroxylase deficiency may affect the ...