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

TITLE=Pediatric adrenocortical carcinoma

JOURNAL=Frontiers in Endocrinology

VOLUME=13

YEAR=2022

URL=https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2022.961650

DOI=10.3389/fendo.2022.961650

ISSN=1664-2392

ABSTRACT=<p>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 <italic>TP53</italic> 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.</p>