AUTHOR=Lalli Enzo , Figueiredo Bonald C. 

TITLE=Pediatric Adrenocortical Tumors: What They Can Tell Us on Adrenal Development and Comparison with Adult Adrenal Tumors

JOURNAL=Frontiers in Endocrinology

VOLUME=6

YEAR=2015

URL=https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2015.00023

DOI=10.3389/fendo.2015.00023

ISSN=1664-2392

ABSTRACT=<p>Adrenocortical tumors (ACT) in children are very rare and are most frequently diagnosed in the context of the Li-Fraumeni syndrome, a multiple cancer syndrome linked to germline mutations of the tumor suppressor gene <italic>TP53</italic> with loss of heterozygosity in the tumors. A peak of children ACT incidence is present in the states of southern Brazil, where they are linked to the high prevalence in the population of a specific <italic>TP53</italic> mutation (R337H). Children ACT have specific features distinguishing them from adult tumors in their pathogenetic mechanisms, genomic profiles, and prognosis. Epidemiological and molecular evidence suggests that in most cases they are derived from the fetal adrenal.</p>