Multiple endocrine neoplasia type 2 risk factors

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [3]

Overview

Common risk factor in the development of multiple endocrine neoplasia type 2 is family history.

Risk Factors

• Family history is the common risk factor in the development of multiple endocrine neoplasia type 2.
• During the Seventh International Multiple Endocrine Neoplasia Meeting in Gubbio in 1999, the risk of medullary thyroid cancer has been stratified in three categories according to the mutations of c-RET as following.

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/

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