Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Childhood Adrenocortical Carcinoma

  • Raul C. Ribeiro
  • Carlos Rodriguez-Galindo
  • Gerard P. Zambetti
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_1088-2


Adrenocortical carcinoma (ACC) is a cancer of the cortex of the adrenal gland (endocrine-related cancers). There are two types. In one type, the tumor continues to secrete the hormones normally produced by the cortex, including glucocorticoids, mineralocorticoids, and adrenal sex hormones. However, these steroids may be produced in excessive amounts, with negative effects on the body. In the other type, the tumor does not produce these hormones and may go undiscovered until it metastasizes.



Cancer of the adrenal cortex is exceedingly rare: only about 300 cases are diagnosed in the United States each year. Adrenocortical tumors (ACT) represent only about 0.2 % of all malignancies in children. The frequency of ACT is 0.4 per million during the first 4 years of life, 0.1 per million during the subsequent 10 years, and 0.2 per million during the late teens. These tumors also occur in adults, usually during the fourth to fifth decades of life. The...


Congenital Adrenal Hyperplasia Adrenocortical Carcinoma Zona Glomerulosa Complete Tumor Resection Zona Fasciculata 
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Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Raul C. Ribeiro
    • 1
  • Carlos Rodriguez-Galindo
    • 2
  • Gerard P. Zambetti
    • 3
  1. 1.Department of OncologySt. Jude Children’s Research HospitalMemphisUSA
  2. 2.Dana-Farber Cancer InstituteBostonUSA
  3. 3.Department of BiochemistryDana-Farber Cancer InstituteBostonUSA