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Pediatric adrenocortical carcinoma.

Maran Ilanchezhian1, Diana Grace Varghese2, John W Glod1

  • 1Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD, United States.

Frontiers in Endocrinology
|November 17, 2022
PubMed
Summary

Pediatric adrenocortical carcinoma (ACC) is rare but more common in Brazil due to Li-Fraumeni syndrome. Current treatments are adapted from adults, with limited research on novel pediatric ACC therapies.

Keywords:
adrenal tumoradrenocortical cancer (ACC)endocrine tumorspediatric adrenal tumorspediatric adrenocortical carcinoma

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Area of Science:

  • Pediatric Endocrinology
  • Oncology
  • Genetics

Background:

  • Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with poor prognosis.
  • Pediatric ACC is exceptionally rare, with higher incidence in Southern Brazil linked to Li-Fraumeni syndrome and TP53 mutations.
  • Li-Fraumeni and Beckwith-Wiedemann syndromes are primary associations in pediatric ACC.

Purpose of the Study:

  • To review the current understanding of pediatric adrenocortical carcinoma.
  • To highlight the unique epidemiological and etiological factors in pediatric ACC.
  • To discuss existing and emerging treatment modalities for pediatric ACC.

Main Methods:

  • Literature review of pediatric adrenocortical carcinoma.
  • Analysis of epidemiological data, focusing on regional variations.
  • Summary of current and investigational therapeutic approaches.

Main Results:

  • Pediatric ACC incidence is low globally but elevated in Southern Brazil.
  • TP53 mutations associated with Li-Fraumeni syndrome drive higher ACC rates in Brazil.
  • Standard treatment involves surgery and adult-derived chemotherapy (EDP) with mitotane.

Conclusions:

  • Pediatric ACC requires tailored research and treatment strategies.
  • Novel therapies like pembrolizumab, cabozantinib, and CAR-T cell therapy warrant further investigation in pediatric ACC.
  • Understanding genetic predispositions is crucial for managing pediatric ACC.