Adrenal cortical neoplasms in children: why so many carcinomas and yet so many survivors?

Pediatr Dev Pathol. Jul-Aug 2009;12(4):284-91. doi: 10.2350/08-06-0489.1.


Adrenal cortical neoplasms in children are represented by a disproportionate number of cases that have been diagnosed pathologically as adrenocortical carcinomas (ACCs)-as many as 90% of all cortical tumors in some pediatric series. Like other solid malignancies of childhood, over half of ACCs present in the first 4 years of life in over 50% of cases. Most are sporadically occurring neoplasms, but ACCs are a manifestation of Beckwith-Wiedemann and Li-Fraumeni syndromes. Despite the fact that the microscopic features are often quite atypical and identical in many respects to ACCs in adults, the clinical outcome is favorable in 70% or more of cases. Tumor weight is seemingly a significant determinant in prognosis at a threshold of greater than 400 g. A risk assessment system is proposed that incorporates tumor weight, localization of tumor to the gland without invasion into the surrounding tissues or organs, and absence of metastasis.

MeSH terms

  • Adrenal Cortex Neoplasms / genetics
  • Adrenal Cortex Neoplasms / mortality
  • Adrenal Cortex Neoplasms / pathology*
  • Adrenocortical Carcinoma / genetics
  • Adrenocortical Carcinoma / mortality
  • Adrenocortical Carcinoma / pathology*
  • Beckwith-Wiedemann Syndrome / genetics
  • Beckwith-Wiedemann Syndrome / pathology
  • Child, Preschool
  • Humans
  • Infant
  • Infant, Newborn
  • Li-Fraumeni Syndrome / genetics
  • Li-Fraumeni Syndrome / pathology
  • Neoplasm Staging
  • Prognosis
  • Risk Assessment
  • Survivors