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

Definition

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.

Characteristics

Incidence

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...

Keywords

Congenital Adrenal Hyperplasia Adrenocortical Carcinoma Zona Glomerulosa Complete Tumor Resection Zona Fasciculata 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Michalkiewicz E, Sandrini R, Figueiredo B et al (2004) Clinical and outcome characteristics of children with adrenocortical tumors: a report from the International Pediatric Adrenocortical Tumor Registry. J Clin Oncol 22:838–845CrossRefPubMedGoogle Scholar
  2. Ribeiro RC, Sandrini F, Figueiredo B et al (2001) An inherited p53 mutation that contributes in a tissue-specific manner to pediatric adrenal cortical carcinoma. Proc Natl Acad Sci U S A 98:9330–9335CrossRefPubMedPubMedCentralGoogle Scholar
  3. Pinto EM, Chen X, Easton J, Finkelstein D, Liu Z, Pounds S, Rodriguez-Galindo C, Lund TC, Mardis ER, Wilson RK, Boggs K, Yergeau D, Cheng J, Mulder HL, Manne J, Jenkins J, Mastellaro MJ, Figueiredo BC, Dyer MA, Pappo A, Zhang J, Downing JR, Ribeiro RC, Zambetti GP (2015) Genomic landscape of paediatric adrenocortical tumours. Nat Commun 6:6302. doi:10.1038/ncomms7302Google Scholar
  4. Pinto EM, Morton C, Rodriguez-Galindo C, McGregor L, Davidoff AM, Mercer K, Debelenko LV, Billups C, Ribeiro RC, Zambetti GP (2013) Establishment and characterization of the first pediatric adrenocortical carcinoma xenograft model identifies topotecan as a potential chemotherapeutic agent. Clin Cancer Res 19(7):1740–7Google Scholar
  5. Ribeiro RC, Pinto EM, Zambetti GP, Rodriguez-Galindo C (2012) The International Pediatric Adrenocortical Tumor Registry initiative: contributions to clinical, biological, and treatment advances in pediatric adrenocortical tumors. Mol Cell Endocrinol 351(1):37–43Google Scholar

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