| ACTH-independent Cushing's syndrome with bilateral micronodular adrenal hyperplasia and ectopic adrenocortical adenoma. | |
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MedLine Citation:
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PMID: 19915020 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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CONTEXT: Bilateral micronodular adrenal hyperplasia and ectopic adrenocortical adenoma are two rare causes of ACTH-independent Cushing's syndrome. OBJECTIVE: The aim of the study was to evaluate a 35-yr-old woman with ACTH-independent hypercortisolism associated with both micronodular adrenal hyperplasia and ectopic pararenal adrenocortical adenoma. DESIGN AND SETTING: In vivo and in vitro studies were performed in a University Hospital Department and academic research laboratories. INTERVENTION: Mutations of the PRKAR1A, PDE8B, and PDE11A genes were searched for in leukocytes and adrenocortical tissues. The ability of adrenal and adenoma tissues to synthesize cortisol was investigated by immunohistochemistry, quantitative PCR, and/or cell culture studies. MAIN OUTCOME MEASURE: Detection of 17alpha-hydroxylase and 21-hydroxylase immunoreactivities, quantification of CYP11B1 mRNA in adrenal and adenoma tissues, and measurement of cortisol levels in supernatants by radioimmunological assays were the main outcomes. RESULTS: Histological examination of the adrenals revealed nonpigmented micronodular cortical hyperplasia associated with relative atrophy of internodular cortex. No genomic and/or somatic adrenal mutations of the PRKAR1A, PDE8B, and PDE11A genes were detected. 17alpha-Hydroxylase and 21-hydroxylase immunoreactivities as well as CYP11B1 mRNA were detected in adrenal and adenoma tissues. ACTH and dexamethasone activated cortisol secretion from adenoma cells. The stimulatory action of dexamethasone was mediated by a nongenomic effect involving the protein kinase A pathway. CONCLUSION: This case suggests that unknown molecular defects can favor both micronodular adrenal hyperplasia and ectopic adrenocortical adenoma associated with Cushing's syndrome. |
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Authors:
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Estelle Louiset; Françoise Gobet; Rossella Libé; Anelia Horvath; Sylvie Renouf; Juliette Cariou; Anya Rothenbuhler; Jérôme Bertherat; Eric Clauser; Philippe Grise; Constantine A Stratakis; Jean-Marc Kuhn; Hervé Lefebvre |
Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't Date: 2009-11-13 |
Journal Detail:
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Title: The Journal of clinical endocrinology and metabolism Volume: 95 ISSN: 1945-7197 ISO Abbreviation: J. Clin. Endocrinol. Metab. Publication Date: 2010 Jan |
Date Detail:
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Created Date: 2010-01-08 Completed Date: 2010-02-01 Revised Date: 2011-07-19 |
Medline Journal Info:
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Nlm Unique ID: 0375362 Medline TA: J Clin Endocrinol Metab Country: United States |
Other Details:
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Languages: eng Pagination: 18-24 Citation Subset: AIM; IM |
Affiliation:
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Institut National de la Santé et de la Recherche Médicale, Unité 982/Equipe Associée 4310, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institut Fédératif de Recherches Multidisciplinaires sur les Peptides 23, University of Rouen, 76821 Mont-Saint-Aignan, France. |
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| MeSH Terms | |
Descriptor/Qualifier:
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Adrenal Cortex Neoplasms
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complications*,
genetics,
radiography Adrenal Glands / pathology*, radiography Adrenocortical Adenoma / complications*, genetics, radiography Adrenocorticotropic Hormone / metabolism, pharmacology Adult Choristoma / complications*, genetics, radiography Cushing Syndrome / complications*, genetics, radiography Drug Resistance / genetics, physiology Female Humans Hyperplasia / complications, genetics, radiography Kidney Diseases / complications, genetics, radiography |
| Chemical | |
Reg. No./Substance:
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9002-60-2/Adrenocorticotropic Hormone |
| Comments/Corrections | |
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