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ACTH-Independent Cushing’s Syndrome: Bilateral Macronodular Hyperplasia

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Cushing's Syndrome

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

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Summary

ACTH-independent macronodular adrenal hyperplasia (AIMAH) is an infrequent cause of Cushing’s syndrome. AIMAH presents as incidental radiological finding or with subclinical or overt Cushing’s syndrome, occasionally with secretion of mineralocorticoids or sex steroids. The pathophysiology of this entity is heterogeneous. The aberrant adrenal expression and function of one or several G-protein coupled receptors can lead to cell proliferation and abnormal regulation of steroidogenesis. In familial cases of AIMAH, specific aberrant hormone receptors may be functional in the adrenal of affected members. Somatic genetic events related to cell cycle regulation, adhesion, and transcription factors occur in addition in the various nodules. Other mechanisms such as Gsp or ACTH receptor mutations and paracrine adrenal hormonal secretions have been rarely identified in other cases of AIMAH. The identification of aberrant receptors can offer specific pharmacological approach to prevent disease progression and control abnormal steroidogenesis alternatively to the usual unilateral or bilateral adrenalectomy.

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Acknowledgments

This work was supported by grant MT-13–189 from Canadian Institutes of Health Research.

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Authors and Affiliations

  1. Division of Endocrinology, Department of Medicine, Centre hospitalier de l’Université de Montréal (CHUM), Montréal, Canada

    Isabelle Bourdeau MD, Antoine Lampron MD, Tânia Longo Mazzuco MD & André Lacroix MD (Professor of Medicine)

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  3. Tânia Longo Mazzuco MD
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  4. André Lacroix 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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Bourdeau, I., Lampron, A., Mazzuco, T.L., Lacroix, A. (2010). ACTH-Independent Cushing’s Syndrome: Bilateral Macronodular Hyperplasia. In: Bronstein, M. (eds) Cushing's Syndrome. Contemporary Endocrinology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60327-449-4_17

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  • DOI: https://doi.org/10.1007/978-1-60327-449-4_17

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