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Review
. 2022 Oct 31:13:961650.
doi: 10.3389/fendo.2022.961650. eCollection 2022.

Pediatric adrenocortical carcinoma

Maran Ilanchezhian  1 , Diana Grace Varghese  2 , John W Glod  1 , Karlyne M Reilly  1 , Brigitte C Widemann  1 , Yves Pommier  2 , Rosandra N Kaplan  1 , Jaydira Del Rivero  2
Affiliations
Review

Pediatric adrenocortical carcinoma

Maran Ilanchezhian et al. Front Endocrinol (Lausanne). .

Abstract

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.

Keywords: adrenal tumor; adrenocortical cancer (ACC); endocrine tumors; pediatric adrenal tumors; pediatric adrenocortical carcinoma.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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