| Adrenal involvement in MEN1. Analysis of 715 cases from the Groupe d'etude des Tumeurs Endocrines database. | |
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MedLine Citation:
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PMID: 22084155 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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AbstractObjectiveLimited data regarding adrenal involvement in MEN1 is available. We describe the characteristics of MEN1-associated adrenal lesions in a large cohort to provide a rationale for their management.MethodsAnalysis of records from 715 MEN1 patients from a multicenter database between 1956 and 2008. Adrenal lesions were compared to those from a multicenter cohort of 144 patients with adrenal sporadic incidentalomas.ResultsAdrenal enlargement was reported in 20.4% (146/715) of patients. Adrenal tumours (> 10 mm in size) accounted for 58.1% of these cases (10.1% of the whole patient cohort). Tumours were bilateral and > 40 mm in size in 12.5% and 19.4% of cases respectively. Hormonal hypersecretion was restricted to patients with tumours and occurred in 15.3% of them. Compared to incidentalomas, MEN1 related tumours exhibited more cases of primary hyperaldosteronism, fewer pheochromocytomas and more adrenocortical carcinomas (ACCs) (13.8% vs 1.3%). Ten ACCs occurred in 8 patients. Interestingly, ACCs occurred after several years of follow-up of small adrenal tumours in 2 of 8 affected patients. Nine of the 10 ACCs displayed ENS@T stage I or II. No evident genotype/phenotype correlation was found for the occurrence of adrenal lesions, endocrine hypersecretion or ACC.ConclusionsAdrenal pathology in MEN1 differs from that observed in sporadic incidentalomas. In the absence of relevant symptoms, endocrine biology can be restricted to patients with adrenal tumours and should focus on steroid secretion including the aldosterone-renin system. MEN1 is a high-risk condition for the occurrence of ACCs. It should be considered regardless of the size of the tumour. |
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Authors:
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Blandine Gatta; Olivier Chabre; Arnaud Murat; Patricia Niccoli; Catherine Bauters; Vincent Rohmer; Jacques Young; Brigitte Delemer; Helene Du Boullay; Marie France Verger; Jean-Marc Kuhn; Jean Louis Sadoul; Philippe Ruszniewski; Albert Beckers; Maud Monsaingeon; Eric Baudin; Pierre Goudet; Antoine Tabarin |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-11-14 |
Journal Detail:
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Title: European journal of endocrinology / European Federation of Endocrine Societies Volume: - ISSN: 1479-683X ISO Abbreviation: - Publication Date: 2011 Nov |
Date Detail:
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Created Date: 2011-11-15 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9423848 Medline TA: Eur J Endocrinol Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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B Gatta, Service d'Endocrinologie, Hôpital Haut Lévêque, Centre Hospitalier Universitaire de Bordeaux, Pessac, France. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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