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Childhood Adrenocortical Carcinoma

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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...

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References

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Correspondence to Raul C. Ribeiro .

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© 2014 Springer-Verlag Berlin Heidelberg

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Ribeiro, R.C., Rodriguez-Galindo, C., Zambetti, G.P. (2014). Childhood Adrenocortical Carcinoma. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-27841-9_1088-2

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  • DOI: https://doi.org/10.1007/978-3-642-27841-9_1088-2

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Online ISBN: 978-3-642-27841-9

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