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Adrenocortical Carcinoma pp 153–172Cite as

Overview of Genetic Syndromes Associated with Adrenocortical Cancer

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Abstract

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).

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  1. Department of Internal Medicine – Division of Metabolism, Endocrinology & Diabetes, University of Michigan Health System, University of Michigan, Domino’s Farms, Lobby C, Suite 1300, 24 Frank Lloyd Wright Drive, PO Box 451, Ann Arbor, MI, 48106, USA

    Tobias Else

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  1. Zina Pitcher Place 109, Ann Arbor, 48109-2200, Michigan, USA

    Gary D. Hammer

  2. Dept. Internal Medicine, Div. Pulmonary & Critical Care Medicine, University of Michigan Health System, Zina Pitcher Place 109, Ann Arbor, 48109-2200, Michigan, USA

    Tobias Else

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Else, T. (2009). Overview of Genetic Syndromes Associated with Adrenocortical Cancer. In: Hammer, G., Else, T. (eds) Adrenocortical Carcinoma. Springer, New York, NY. https://doi.org/10.1007/978-0-387-77236-3_10

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