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Pituitary Disease in MEN Type 1 (MEN1): Data from the France-Belgium MEN1 Multicenter Study

Department of Endocrinology (B.V., F.B.), University Hospital, 21000 Dijon, France; Department of Surgery (P.G., P.C.), University Hospital, 21000 Dijon, France; Department of Endocrinology (A.M.), University Hospital, 44000 Nantes, France; Department of Endocrinology (A.B.), Sart Tilman University, 4000 Liège, Belgium; Department of Nuclear Medicine (G.S.), Hôpital Neuro-Cardiologique, 69000 Lyon, France; and Laboratory of Genetics (B.C., C.M., A.C.), Hôpital Edouard Herriot, 69437 Lyon, France

Address all correspondence and requests for reprints to: Bruno Vergès, Service d’Endocrinologie, Hôpital du Bocage, 2, bd Mal de Lattre de Tassigny, B. P. 1542, 21034 Dijon Cedex, France.

Abstract

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.

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