Reference : Pituitary disease in MEN type 1 (MEN1): Data from the France-Belgium MEN1 multicenter...
Scientific journals : Article
Human health sciences : Endocrinology, metabolism & nutrition
http://hdl.handle.net/2268/23756
Pituitary disease in MEN type 1 (MEN1): Data from the France-Belgium MEN1 multicenter study
English
Vergès, B. [ > > ]
Boureille, F. [ > > ]
Goudet, P. [ > > ]
Murat, A. [ > > ]
Beckers, Albert mailto [Université de Liège - ULiège > Département des sciences cliniques > Endocrinologie]
Sassolas, G. [ > > ]
Cougard, P. [ > > ]
Chambe, B. [ > > ]
Montvernay, C. [ > > ]
Calender, A. [ > > ]
Feb-2002
Journal of Clinical Endocrinology and Metabolism
Endocrine Society
87
2
457-465
Yes (verified by ORBi)
International
0021-972X
Chevy Chase
MD
[en] Secretory tumor ; Parathyroid diseases ; Benign neoplasm ; Endocrinopathy ; Europe ; Neuroendocrine tumor ; Gene ; Genetics ; Diagnosis ; Comparative study ; Belgium ; France ; Human ; Adrenocorticotropic hormone ; Female ; Age of onset ; Multicenter study ; Prolactinoma ; Somatotropin hormone ; Hyperparathyroidism ; Adenoma ; Pituitary diseases
[fr] Tumeur sécrétante ; Parathyroïde pathologie ; Tumeur bénigne ; Endocrinopathie ; Europe ; Tumeur neuroendocrinienne ; Gène ; Génétique ; Diagnostic ; Etude comparative ; Belgique ; France ; Homme ; ACTH ; Femelle ; Age apparition ; Etude multicentrique ; Prolactinome ; STH ; Hyperparathyroïdie ; Adénome ; Hypophyse pathologie
[en] To date, data on pituitary adenomas in MEN type 1 (MEN1) still have to be evaluated. We analyzed the data of a large series of 324 MEN1 patients from a French and Belgian multicenter study. Data on pituitary disease were compared with those from 110 non-MEN1 patients with pituitary adenomas, matched for age, year of diagnosis, and follow-up period. Genetic analysis of the MEN1 gene was performed in 197 of the MEN1 patients. In our MEN1 series, pituitary disease occurred in 136 of 324 (42%), less frequently than hyperparathyroidism (95%, P < 0.001) and endocrine enteropancreatic tumors (54%, P < 0.01). Mean age of onset of pituitary tumors was 38.0 +/- 15.3 yr (range, 12-83 yr). Pituitary disease was associated with hyperparathyroidism in 90%. of cases, with enteropancreatic tumors in 47%, with adrenal tumors in 16%, and with thoracic neuroendocrine tumors in 4%. Pituitary disease was the initial lesion of MEN1 in 17% of all MEN1 patients. MEN1 pituitary adenomas were significantly more frequent in women than in men (50% vs. 31%,P < 0.001). Among the 136 pituitary adenomas, there were 85 prolactinomas and 12 GH-secreting, 6 ACTH-secreting, 13 cosecreting, and 20 nonsecreting tumors. Eighty-five percent of MEN1-related pituitary lesions were macroadenomas (vs. 42% in non-MEN1 patients, P < 0.001), including 32% of invasive cases. Among secreting adenomas, hormonal hypersecretion was normalized, after treatment, in only 42% (vs. 90% in non-MEN1 patients, P < 0.001), with a median follow-up of 11.4 yr. No correlation was found between the type of MEN1 germ-line mutation and the presence or absence of pituitary adenoma. Our study, based on a large group of MEN1 patients, shows that pituitary adenomas occur in 42% of the cases and are characterized by a larger size and a more aggressive presentation than without MEN1.
Researchers ; Professionals
http://hdl.handle.net/2268/23756
10.1210/jc.87.2.457
Cet article a bénéficié d'un éditorial de : S.J. Marx - NIH Bethesda, Maryland. Agressive Pituitary Tumors in MEN1 : Do they refuse the Two-Hit Model of Tumorigenesis ? Editorial publié dans J.Clin.Endocrinol.Metab, vol 87 ; n°2 February 2002 : 453-456.

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