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ACTH-Independent Cushing’s Syndrome: Adrenocortical Tumors

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Cushing's Syndrome

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

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Summary

Approximately 15–20% of endogenous Cushing’s syndrome (CS) is secondary to adrenal gland disorders. Unilateral adrenocortical tumors (adenoma or carcinomas) are responsible for the majority of the cases of primary adrenal CS. Adrenocortical tumors are more common in the population above 50 years of age. Steroid-secreting adrenal tumors causing endocrine disease are rare, however, they correspond to 80% of all causes of ACTH-independent Cushing’s syndrome. The most common clinical presentation in adults is CS, contrasting with pediatric patients in whom virilization syndrome is more frequent.

The incidence of adrenocortical carcinoma in the United States is estimated to be 0.2–0.4 per million population per year. Nevertheless, there is a high incidence of adrenocortical tumors in Southern and Southwestern region of Brazil, estimated to be approximately 10–15 times greater than the worldwide incidence.

In this chapter, we review the clinical features, hormonal and imaging diagnosis, and treatment of ACTH-independent Cushing’s syndrome due to adrenocortical tumors.

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Authors and Affiliations

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

    Maria Candida Barisson Villares Fragoso MD

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

    Sorahia Domenice MD

  3. 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)

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

    Berenice Bilharinho Mendonca MD

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  1. Maria Candida Barisson Villares Fragoso MD
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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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Fragoso, M.C.B.V., Domenice, S., Latronico, A.C., Mendonca, B.B. (2010). ACTH-Independent Cushing’s Syndrome: 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_16

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