Clinical prognostic factors in pediatric adrenocortical tumors: A meta-analysis

Pediatr Blood Cancer. 2021 Mar;68(3):e28836. doi: 10.1002/pbc.28836. Epub 2020 Dec 11.


Pediatric adrenocortical tumors (ACT) are rare and sometimes aggressive malignancies, but there is no consensus on the outcome predictors in children. A systematic search of MEDLINE, SCOPUS, Web of Science, and the Cochrane Library for studies from 1994 to 2020 about pediatric ACT was performed. In 42 studies, 1006 patients, aged 0-18 years, were included. The meta-analyses resulted in the following predictors of better outcome: age <4 years (P < .00001), nonsecreting tumors (P = .004), complete surgical resection (P < .00001), tumor volume (P < .0001), tumor weight (P < .00001), tumor maximum diameter (P = .0009), and Stage I disease (P < .00001). Moreover, patients affected by Cushing syndrome showed a worse outcome (P < .0001). International prospective studies should be implemented to standardize clinical prognostic factors evaluation, together with pathological scores, in the stratification of pediatric ACT.

Keywords: adrenocortical carcinoma; adrenocortical tumors; children; meta-analysis; prognosis study; prognostic factors; systematic review.

Publication types

  • Meta-Analysis

MeSH terms

  • Adrenal Cortex Neoplasms / mortality*
  • Adrenal Cortex Neoplasms / pathology
  • Adrenal Cortex Neoplasms / therapy
  • Adrenocortical Carcinoma / mortality*
  • Adrenocortical Carcinoma / pathology
  • Adrenocortical Carcinoma / therapy
  • Child
  • Humans
  • Prognosis
  • Survival Rate