RT Book, Section A1 Whaley, J. Greg A1 Lairmore, Terry C. A2 Morita, Shane Y. A2 Dackiw, Alan P. B. A2 Zeiger, Martha A. SR Print(0) ID 6163914 T1 Chapter 21. Multiple Endocrine Neoplasia Type 1 T2 McGraw-Hill Manual: Endocrine Surgery YR 2010 FD 2010 PB The McGraw-Hill Companies PP New York, NY SN 978-0-07-160645-5 LK accesssurgery.mhmedical.com/content.aspx?aid=6163914 RD 2024/03/29 AB Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant genetic predisposition to develop multiglandular parathyroid disease, benign and malignant neuroendocrine tumors (NET of the pancreas and duodenum, and adenomas of the anterior pituitary.1,2 Benign and malignant tumors of these organs may develop, with multiple target organs affected and multifocal tumors within a target organ. Less often, carcinoid tumors (bronchial, gastrointestinal, or thymic), lipomas, cutaneous angiofibromas, or ependymomas of the central nervous system may be associated with this syndrome. The clinical definition of MEN1 includes a patient with tumor development in two associated endocrine tissues (parathyroid, pancreas, pituitary) and a first-degree relative with involvement of one of the three principal endocrine tissues. Direct genetic testing can detect disease-associated germline mutations in the MEN1 gene and identify affected individuals.