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Jeffrey A. Norton, Tony D. Fang, and Robert T. Jensen

cloning of the gene for multiple endocrine neoplasia-type 1 . Science 1997 ; 276 : 404 – 407 . 2. Schussheim DH Skarulis MC Agarwal SK . Multiple endocrine neoplasia type 1: new clinical and basic findings . Trends Endocrinol Metab 2001

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Jeffrey F. Moley

in all patients with multiple endocrine neoplasia type 2 (MEN 2) syndromes (25% of cases). Second, although MTC is more aggressive than differentiated thyroid carcinoma, it is still an indolent malignancy, with reported 10-year survival rates from 69

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Jennifer B. Ogilvie and Electron Kebebew

interest, arrangement, or affiliation with the manufacturers of any products discussed in the article or their competitors. References 1. Gagel RF Marx SJ . Multiple endocrine neoplasia . In: Larsen PR, Kronenberg HM, Melmed S, et al., eds

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Manisha H. Shah, Whitney S. Goldner, Al B. Benson III, Emily Bergsland, Lawrence S. Blaszkowsky, Pamela Brock, Jennifer Chan, Satya Das, Paxton V. Dickson, Paul Fanta, Thomas Giordano, Thorvardur R. Halfdanarson, Daniel Halperin, Jin He, Anthony Heaney, Martin J. Heslin, Fouad Kandeel, Arash Kardan, Sajid A. Khan, Boris W. Kuvshinoff II, Christopher Lieu, Kimberly Miller, Venu G. Pillarisetty, Diane Reidy, Sarimar Agosto Salgado, Shagufta Shaheen, Heloisa P. Soares, Michael C. Soulen, Jonathan R. Strosberg, Craig R. Sussman, Nikolaos A. Trikalinos, Nataliya A. Uboha, Namrata Vijayvergia, Terence Wong, Beth Lynn, and Cindy Hochstetler

the United States may exceed 170,000. Most NETs seem to be sporadic, and risk factors for sporadic NETs are poorly understood. NETs may also arise in the context of inherited genetic syndromes, including multiple endocrine neoplasia types 1 (MEN1), 2

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R. Michael Tuttle, Douglas W. Ball, David Byrd, Gilbert H. Daniels, Raza A. Dilawari, Gerard M. Doherty, Quan-Yang Duh, Hormoz Ehya, William B. Farrar, Robert I. Haddad, Fouad Kandeel, Richard T. Kloos, Peter Kopp, Dominick M. Lamonica, Thom R. Loree, William M. Lydiatt, Judith McCaffrey, John A. Olson Jr., Lee Parks, John A. Ridge, Jatin P. Shah, Steven I. Sherman, Cord Sturgeon, Steven G. Waguespack, Thomas N. Wang, and Lori J. Wirth

neuroendocrine parafollicular calcitonin-producing (C) cells of the thyroid. 2 – 4 Sporadic MTC accounts for approximately 80% of all cases of the disease. The remaining cases consist of inherited tumor syndromes, such as multiple endocrine neoplasia type 2A

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Stephanie A. Terezakis and Nancy Y. Lee

MTC accounts for approximately 4% of thyroid cancers. Both familial (FMTC) and sporadic forms of MTC exist; FMTC is associated with the autosomal dominant hereditary syndromes, including multiple endocrine neoplasia (MEN) type 2A, MEN type 2B, and non

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Maria A. Kouvaraki, Suzanne E. Shapiro, Jeffrey E. Lee, Douglas B. Evans, and Nancy D. Perrier

through 1970 . Mayo Clin Proc 1992 ; 67 : 934 – 940 . 57 Wells SA Jr Chi DD Toshima K . Predictive DNA testing and prophylactic thyroidectomy in patients at risk for multiple endocrine neoplasia type 2A . Ann Surg 1994 ; 220 : 237 – 247

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Ariel Polish, Maxwell T. Vergo, and Mark Agulnik

) Pheochromocytomas/paragangliomas Poorly differentiated (high-grade or anaplastic or anaplastic/small cell tumors) Multiple endocrine neoplasia type 1 Multiple endocrine neoplasia type 2 Functional NETs can cause symptoms from the release of hormones, whereas

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Jonathan R. Strosberg, Gary D. Hammer, and Gerard M. Doherty

Edited by Kerrin G. Robinson

2001 ; 86 : 5658 – 5671 . 10 Marx S Spiegel AM Skarulis MC . Multiple endocrine neoplasia type 1: clinical and genetic topics . Ann Intern Med 1998 ; 129 : 484 – 494 . 11 Soon PS McDonald KL Robinson BG Sidhu SB . Molecular

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David S. Ettinger, Gregory J. Riely, Wallace Akerley, Hossein Borghaei, Andrew C. Chang, Richard T. Cheney, Lucian R. Chirieac, Thomas A. D’Amico, Todd L. Demmy, Ramaswamy Govindan, Frederic W. Grannis Jr, Stefan C. Grant, Leora Horn, Thierry M. Jahan, Ritsuko Komaki, Feng-Ming (Spring) Kong, Mark G. Kris, Lee M. Krug, Rudy P. Lackner, Inga T. Lennes, Billy W. Loo Jr, Renato Martins, Gregory A. Otterson, Jyoti D. Patel, Mary C. Pinder-Schenck, Katherine M. Pisters, Karen Reckamp, Eric Rohren, Theresa A. Shapiro, Scott J. Swanson, Kurt Tauer, Douglas E. Wood, Stephen C. Yang, Kristina Gregory, and Miranda Hughes

for Neuroendocrine Tumors (available at NCCN.org ); they are associated with multiple endocrine neoplasia type 1 syndrome (MEN1). 11 , 12 Lung carcinoids are discussed in the NCCN Guidelines for Small Cell Lung Cancer (see “Lung Neuroendocrine Tumors