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Adrenocortical Cancer

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Encyclopedia of Cancer

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Synonyms

Carcinoma of the adrenal cortex; Malignant adrenocortical tumor

Definition

Adrenocortical cancer (ACC) is a malignant tumor from the adrenal cortex. It is a rare tumor with a poor prognosis. The consequences of ACC are due to tumor growth and metastasis and also due to steroid oversecretion.

Characteristics

Epidemiology of Adrenocortical Cancer

ACC is a rare disease with an estimated incidence between 1 and 2 per million and per year in adults in North America and Europe.

Pathophysiology of Adrenocortical Cancer

Analysis of the pattern of X-chromosome inactivation in heterozygous female tissue has shown that ACC consists of monoclonal populations of cells. A large number of molecular techniques, such as comparative genomic hybridization (CGH) and microsatellite analysis, have identified alterations affecting various chromosomes and loci in ACC. Most of the changes observed concern losses on chromosomes 2, 11q and 17p, and gains on chromosomes 4 and 5. Studies using...

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References

  • Allolio B, Hahner S, Weismann D et al (2004) Management of adrenocortical carcinoma. Clin Endocrinol (Oxf) 60(3):273–287

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  • Bertherat J, Groussin L, Bertagna X (2006) Mechanisms of disease: adrenocortical tumors – molecular advances and clinical perspectives. Nat Clin Pract Endocrinol Metab 2(11):632–641

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  • Giordano TJ, Thomas DG, Kuick R et al (2003) Distinct transcriptional profiles of adrenocortical tumors uncovered by DNA microarray analysis. Am J Pathol 162(2):521–531

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  • Libe R, Bertherat J (2005) Molecular genetics of adrenocortical tumours, from familial to sporadic diseases. Eur J Endocrinol 153(4):477–487

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  • Sidhu S, Sywak M, Robinson B et al (2004) Adrenocortical cancer: recent clinical and molecular advances. Curr Opin Oncol 16(1):13–18

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Author information

Authors and Affiliations

  1. Endocrinology, Metabolism and Cancer Department, INSERM U567, Institut Cochin, Paris, France

    Rossella Libè & Jérôme Bertherat

Authors
  1. Rossella Libè
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  2. Jérôme Bertherat
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Corresponding author

Correspondence to Rossella Libè .

Editor information

Editors and Affiliations

  1. Abt. Tumorgenetik, Deutsches Krebsforschungszentrum, Heidelberg, Germany

    Manfred Schwab

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© 2014 Springer-Verlag Berlin Heidelberg

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Libè, R., Bertherat, J. (2014). Adrenocortical Cancer. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-27841-9_120-2

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  • DOI: https://doi.org/10.1007/978-3-642-27841-9_120-2

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Online ISBN: 978-3-642-27841-9

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