TP53 and Li-Fraumeni syndrome: TP53, located within 17p13, plays a pivotal role in regulating various cellular processes, including cellular proliferation, DNA repair, and apoptosis.11 Approximately 70% of germline mutations in TP53 are associated with Li-Fraumeni syndrome, which predisposes individuals to various cancers, including ACC.11 Southern Brazil exhibits a higher incidence of adrenocortical tumors, especially in patients in pediatric care, linked to a higher prevalence of TP53 germline mutations.23 In sporadic ACC cases, somatic TP53 mutations are found in approximately 25% of instances.24,25