Multiple endocrine neoplasia type 2 risk factors

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]

Overview

Family history is the most common risk factor in the development of multiple endocrine neoplasia type 2.

Family history is the most common risk factor in the development of multiple endocrine neoplasia type 2.^[1]^[2]^[3]^[4]
In 1999, during the Seventh International Multiple Endocrine Neoplasia Meeting in Gubbio , the risk of medullary thyroid cancer has been stratified in three categories according to the mutations of c-RET as following.^[5]^[3]

**Risk Stratification**
Gene	Risk
Children with multiple endocrine neoplasia type 2B and/or c-RET codon 883, 918, 922	Highest risk of aggressive medullary thyroid cancer
Children with any c-RET codon 611, 618, 620 or 634 mutations	High risk of medullary thyroid cancer
Children with c-RET codon 609, 768, 790, 791, 804 and 891 mutations	Less aggressive and slowly growing medullary thyroid cancer
Adapted from Marquard J, Eng C. Multiple Endocrine Neoplasia Type 2. 1999 Sep 27 [Updated 2015 Jun 25]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2015. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1257/^[6]

↑ Yip, Linwah (2003). "Multiple Endocrine Neoplasia Type 2". Archives of Surgery. 138 (4): 409. doi:10.1001/archsurg.138.4.409. ISSN 0004-0010.
↑ Multiple endocrine neoplasia type2. Orphanet (30.10.2015). http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=653 Accessed on October, 30, 2015
↑ ^3.0 ^3.1 Marini, Francesca; Falchetti, Alberto; Del Monte, Francesca; Carbonell Sala, Silvia; Tognarini, Isabella; Luzi, Ettore; Brandi, Maria (2006). Orphanet Journal of Rare Diseases. 1 (1): 45. doi:10.1186/1750-1172-1-45. ISSN 1750-1172. Missing or empty |title= (help)
↑ Friedhelm Raue & Karin Frank-Raue (2012). "Genotype-phenotype correlation in multiple endocrine neoplasia type 2". Clinics (Sao Paulo, Brazil). 67 Suppl 1: 69–75. doi:10.6061/clinics/2012(sup01)13. PMID 22584709.
↑ Liu, Qiuli; Tong, Dali; Yuan, Wenqiang; Liu, Gaolei; Yuan, Gang; Lan, Weihua; Zhang, Dianzheng; Zhang, Jun; Huang, Zaoming; Zhang, Yao; Jiang, Jun (2017). "Different RET gene mutation-induced multiple endocrine neoplasia type 2A in 3 Chinese families". Medicine. 96 (3): e5967. doi:10.1097/MD.0000000000005967. ISSN 0025-7974.
↑ Jessica Marquard & Charis Eng (1993). "Multiple Endocrine Neoplasia Type 2". PMID 020301434.