Overview of Genetic Syndromes Associated with Adrenocortical Cancer

  • Tobias Else


The volume of literature on the association of benign and malignant adrenocortical neoplasms with genetic syndromes is overwhelming. However, inherent problems arise when attempting to determine the specificity of these associations. On one hand, it is difficult to establish a clear link between the common benign adrenocortical adenomas and the rare congenital syndromes, and one must exercise caution when interpreting these observations. An exception, of course, is the case in which a specific phenotype of functional hormone-secreting lesions is observed. For example, diagnosis may be easier with rare syndromes, such as McCune-Albright syndrome, or with a specific diagnostic response to a diagnostic test as in Carney complex (i.e., the paradox of increased cortisol secretion after dexamethasone application).


Familial Adenomatous Polyposis Lynch Syndrome Adrenal Lesion Familial Adenomatous Polyposis Patient Adrenocortical Tumor 
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© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  1. 1.Department of Internal Medicine – Division of Metabolism, Endocrinology & DiabetesUniversity of Michigan Health System, University of MichiganAnn ArborUSA

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