Abstract
Multiple endocrine neoplasia-1 (MEN-1) is an autosomal dominant inherited syndrome that occurs due to inactivating mutations of the MEN1 gene locus, coding for a tumor-suppressor protein, menin. The components of MEN-1 are hyperparathyroidism due to multiple parathyroid adenomas, pancreatic neuroendocrine tumors, and pituitary adenomas, in addition to some less common neoplastic manifestations. Care of people with MEN-1 requires knowledge of the problems that may arise, and the best approaches to detect and care for the manifestations of this incurable, but manageable, disease.
(c) 2005 Wiley-Liss, Inc.
MeSH terms
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Adenoma / diagnosis
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Endosonography
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Female
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Genes, Tumor Suppressor*
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Humans
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Hyperparathyroidism / etiology
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Insulinoma / diagnostic imaging
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Insulinoma / surgery
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Male
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Multiple Endocrine Neoplasia Type 1* / diagnosis
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Multiple Endocrine Neoplasia Type 1* / genetics
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Multiple Endocrine Neoplasia Type 1* / surgery
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Neuroectodermal Tumors / diagnostic imaging
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Pancreatic Neoplasms / diagnostic imaging
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Pancreatic Neoplasms / surgery
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Parathyroid Neoplasms / diagnosis
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Pituitary Neoplasms / surgery
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Prolactinoma / surgery
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Proto-Oncogene Proteins / genetics*
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Tomography, Emission-Computed, Single-Photon
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Tomography, X-Ray Computed
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Vipoma / surgery
Substances
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MEN1 protein, human
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Proto-Oncogene Proteins