Pediatric adrenocortical tumor - review and management update

Autor(es)

Brondani, Vania B. ^[1] ; Fragoso, V, Maria Candida B.

Afiliação do(s) autor(es)

^[1] V, Univ Sao Paulo, Fac Med, Serv Endocrinol & Metabol, Hosp Clin, Unidade Suprarrenal, Lab Hormonios & Gen, Av Dr Eneas de Carvalho Aguiar 155, Sao Paulo - Brazil

• Tipo de documento: Artigo de Revisão
• Fonte: CURRENT OPINION IN ENDOCRINOLOGY DIABETES AND OBESITY; v. 27, n. 3, p. 177-186, JUN 2020.
• Citações Web of Science: 0

Resumo

Purpose of review Adrenocortical tumor (ACT) is a rare disease with an annual worldwide incidence of 0.3-0.38/million children below 15 years old, and Brazilian population presents the highest incidence because of germline mutation in theTP53. Pediatric ACT is associated with virilizing features and hypercortisolism in most cases. Malignancy is defined when local invasion or metastasis is found, and it is associated with a poor prognosis. However, the correct and early diagnosis and treatment may impact on overall and disease-free survival. Recent findings A complete understanding of the disease and its singularities facilitates the assistance to the pediatric patient with ACT. The new insights about adrenal tumorigenesis have provided a better understanding of this disease. In this scenario, the era of molecular studies is leading to the refinement of the taxonomy, and it is offering the opportunity to discover new biomarkers and pathways of tumorigenesis, beyond the knowing beta-catenin, Insulin-like growth factor-II/IGF-IR, and the p53/Rb signaling. The rarity of this disease makes it a real challenge. Here, we present a review focusing on clinical practice. A methodic approach aiming to clarify the diagnosis and a follow-up are suggested to guide physicians in the assistance of pediatrics patients, improving the prognosis. (AU)

• Processo FAPESP: 17/26345-5 - Avaliação de fatores prognósticos dos tumores do córtex da suprarrenal e preditivos da resposta ao tratamento com mitotano em carcinomas
• Beneficiário: Maria Candida Barisson Villares Fragoso
• Modalidade de apoio: Auxílio à Pesquisa - Regular