Pediatric adrenocortical carcinoma

• Published in: Frontiers in endocrinology (Lausanne) Vol. 13; p. 961650
• Main Authors: Ilanchezhian, Maran, Varghese, Diana Grace, Glod, John W, Reilly, Karlyne M, Widemann, Brigitte C, Pommier, Yves, Kaplan, Rosandra N, Del Rivero, Jaydira
• Format: Journal Article
• Language: English
• Published: Switzerland Frontiers Media S.A 31-10-2022
• Subjects: Adrenal Cortex Neoplasms - genetics; Adrenal Cortex Neoplasms - pathology; Adrenal Cortex Neoplasms - therapy; adrenal tumor; adrenocortical cancer (ACC); Adrenocortical Carcinoma - diagnosis; Adrenocortical Carcinoma - genetics; Adrenocortical Carcinoma - therapy; Adult; Child; endocrine tumors; Endocrinology; Humans; Li-Fraumeni Syndrome - genetics; Mitotane - therapeutic use; pediatric adrenal tumors; pediatric adrenocortical carcinoma; Young Adult; adrenal tumor; pediatric adrenal tumors; endocrine tumors; pediatric adrenocortical carcinoma; adrenocortical cancer (ACC)
• ISSN: 1664-2392; 1664-2392
• DOI: 10.3389/fendo.2022.961650

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
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.