Multiple Endocrine Neoplasia Type 1 (MEN1)

Cornelis J. Lips, Gerlof D. Valk, Koen M. Dreijerink, Marc Timmers, Rob B. van der Luijt, Thera P. Links, Bernadette P. M. van Nesselrooij, Menno Vriens, Jo W. Höppener, Inne Borel Rinkes, Anouk N. A. van der Horst-Schrivers

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Abstract

Multiple endocrine neoplasia type 1 is an autosomal dominantly inherited syndrome. MEN1 is characterized by the occurrence of tumors of the parathyroid glands, the pancreatic islets, the anterior pituitary gland, and the adrenal glands, as well as neuroendocrine tumors in the thymus, lungs, and stomach, often at a young age. Nonendocrine manifestations of MEN1 include angiofibromas, collagenomas, lipomas, leiomyomas, and meningiomas. The prevalence of MEN1 is two to three per 100,000, and is equal among males and females. MEN1 and multiple endocrine neoplasia type 2 (MEN2) are two distinct syndromes. In MEN2, patients frequently develop medullary thyroid carcinoma and adrenal medullary tumors (pheochromocytoma).
Original languageEnglish
Title of host publicationGenetic Diagnosis of Endocrine Disorders: Second Edition
PublisherElsevier Inc.
Pages343-359
ISBN (Print)9780128008928
DOIs
Publication statusPublished - 23 Oct 2015
Externally publishedYes

Publication series

NameGenetic Diagnosis of Endocrine Disorders: Second Edition

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