Portal de Periódicos da CAPES

Pediatric adrenocortical carcinoma

2022; Frontiers Media; Volume: 13; Linguagem: Inglês

10.3389/fendo.2022.961650

1664-2392

Maran Ilanchezhian, Diana Grace Varghese, John Glod, Karlyne M. Reilly, Brigitte C. Widemann, Yves Pommier, Rosandra N. Kaplan, Jaydira Del Rivero,

Neuroblastoma Research and Treatments

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy of the adrenal gland with an unfavorable prognosis. It is rare in the pediatric population, with an incidence of 0.2-0.3 patients per million in patients under 20 years old. It is primarily associated with Li-Fraumeni and Beckwith-Wiedemann tumor predisposition syndromes in children. The incidence of pediatric ACC is 10-15fold higher in southern Brazil due to a higher prevalence of TP53 mutation associated with Li-Fraumeni syndrome in that population. Current treatment protocols are derived from adult ACC and consist of surgery and/or chemotherapy with etoposide, doxorubicin, and cisplatin (EDP) with mitotane. Limited research has been reported on other treatment modalities for pediatric ACC, including mitotane, pembrolizumab, cabozantinib, and chimeric antigen receptor autologous cell (CAR-T) therapy.