Pediatric adrenocortical tumours

Best Pract Res Clin Endocrinol Metab. 2020 May;34(3):101448. doi: 10.1016/j.beem.2020.101448. Epub 2020 Jul 3.


Childhood adrenocortical tumors (ACTs) are rare, representing ∼0.2% of all pediatric malignancies and having an incidence of 0.2-0.3 new cases per million per year in the United States, but incidences are remarkably higher in Southern Brazil. At diagnosis, most children show signs and symptoms of virilization, Cushing syndrome, or both. Less than 10% of patients with ACT exhibit no endocrine syndrome at presentation, although some show abnormal concentrations of adrenal cortex hormones. Pediatric ACT is commonly associated with constitutional genetic and/or epigenetic alterations, represented by germline TP53 mutations or chromosome 11p abnormalities. Complete tumor resection is required to achieve cure. The role of chemotherapy is not established, although definitive responses to several anticancer drugs are documented. For patients undergoing complete tumor resection, favorable prognostic factors include young age, small tumor size, virilization, and adenoma histology. Prospective studies are necessary to further elucidate the pathogenesis of ACT and improve patient outcomes.

Keywords: 11p15; TP53; adrenal; carcinoma; pediatric.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adenoma / diagnosis
  • Adenoma / epidemiology*
  • Adenoma / genetics
  • Adenoma / pathology
  • Adrenal Cortex Neoplasms / diagnosis
  • Adrenal Cortex Neoplasms / epidemiology*
  • Adrenal Cortex Neoplasms / genetics
  • Adrenal Cortex Neoplasms / pathology
  • Age of Onset
  • Antineoplastic Agents / therapeutic use
  • Child
  • Child, Preschool
  • Female
  • Germ-Line Mutation
  • Humans
  • Incidence
  • Male
  • Prognosis
  • Prospective Studies
  • Virilism / epidemiology
  • Virilism / etiology


  • Antineoplastic Agents