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Genetics of Endocrine and Neuroendocrine Neoplasias (PDQ®): Genetics - Health Professional Information [NCI] - Introduction

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Germline DNA -based testing of the RET gene (chromosomal region 10q11.2) identifies disease-causing mutations in more than 95% of individuals with MEN2A and MEN2B and in about 88% of individuals with FMTC.[3]

The prevalence of MEN2 has been estimated to be between 1 in 30,000 [4,5] and 1 in 35,000 individuals.[6] The vast majority of MEN2 cases are MEN2A. In the United States, an estimated 451 cases of MEN2-related MTC are diagnosed per year.[7]

References:

  1. Agarwal SK, Ozawa A, Mateo CM, et al.: The MEN1 gene and pituitary tumours. Horm Res 71 (Suppl 2): 131-8, 2009.
  2. Eng C: Seminars in medicine of the Beth Israel Hospital, Boston. The RET proto-oncogene in multiple endocrine neoplasia type 2 and Hirschsprung's disease. N Engl J Med 335 (13): 943-51, 1996.
  3. Brandi ML, Gagel RF, Angeli A, et al.: Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86 (12): 5658-71, 2001.
  4. Mulligan LM, Kwok JB, Healey CS, et al.: Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A. Nature 363 (6428): 458-60, 1993.
  5. Donis-Keller H, Dou S, Chi D, et al.: Mutations in the RET proto-oncogene are associated with MEN 2A and FMTC. Hum Mol Genet 2 (7): 851-6, 1993.
  6. DeLellis RA, Lloyd RV, Heitz PU, et al., eds.: Pathology and Genetics of Tumours of Endocrine Organs. Lyon, France: IARC Press, 2004. World Health Organization classification of tumours, vol. 8.
  7. American Cancer Society.: Cancer Facts and Figures 2013. Atlanta, Ga: American Cancer Society, 2013. Available online. Last accessed March 13, 2013.
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Last Updated: February 25, 2014
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