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Cushing's Syndrome pp 41–52Cite as

Pathogenesis of Adrenocortical Tumors

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Part of the book series: Contemporary Endocrinology ((COE))

Summary

Adrenocortical carcinomas are rare malignant tumors. However, the high incidence of the adrenocortical tumors in pediatric and adult patients from the Southern region of Brazil is particularly remarkable, since it has been estimated to be approximately ten times greater than the worldwide incidence. Adrenocortical tumors occur as a component of several hereditary tumor syndromes, which include the Li–Fraumeni syndrome, Beckwith–Wiedemann syndrome, multiple endocrine neoplasia 1, Carney complex, and congenital adrenal hyperplasia. The study of these rare genetic syndromes has greatly contributed to the elucidation of sporadic adrenocortical tumorigenesis. This chapter summarizes the molecular alterations likely involved in the multistage tumorigenesis of familial or sporadic adrenocortical adenomas and carcinomas.

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Authors and Affiliations

  1. Unidade de Endocrinologia do Desenvolvimento e Laboratório de Hormônios e Genética Molecular LIM-42, Hospital das Clínicas da Faculdade de Medicina da, Universidade de São Paulo, São Paulo, Brasil

    Madson Q. Almeida MD & Emilia M. Pinto MD

  2. Unidade de Endocrinologia do Desenvolvimento e Laboratório de Hormônios e Genética Molecular LIM/42, Hospital das Clínicas da Faculdade de Medicina da, Universidade de São Paulo, Av. Dr. Enéas de carvelho Aguiar, 155, 2 andar Bloco 6, São Paulo, Brasil

    Ana Claudia Latronico (Associate Professor of Medicine)

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  1. Madson Q. Almeida MD
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  2. Emilia M. Pinto MD
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  3. Ana Claudia Latronico
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  1. , Division of Endocrinology and Metabolism, Hospital das Clínicas, University of Sao, Sao Paulo, 05403-000, Brazil

    Marcello D. Bronstein

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Almeida, M.Q., Pinto, E.M., Latronico, A.C. (2010). Pathogenesis of Adrenocortical Tumors. In: Bronstein, M. (eds) Cushing's Syndrome. Contemporary Endocrinology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60327-449-4_4

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