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Genetics of Endocrine and Neuroendocrine Neoplasias (PDQ®)

  • Updated: 07/17/2014

Table 5. American Thyroid Association Medullary Thyroid Cancer Risk Stratification and Management Guidelinesa

Risk level Mutated Codon(s) Age of RET Testing Timing of Prophylactic Thyroidectomy 
D883, 918, and compound heterozygotes V804M+E805K, V804M+Y806C, and V804M+S904CASAP and within the first y of lifeASAP and within the first y of life.
C634<3–5 yBefore age 5 y.
B609b, 611, 618, 620, 630b, and compound heterozygote V804M+V778I<3–5 yConsider surgery before age 5 y. May delay surgery after age 5 y if criteria are met.c
A768, 790, 791, 804, 891<3–5 yMay delay surgery after age 5 y if criteria are met.c

aAdapted from Kloos et al.[25]
bThese mutations had not been reported at the time of the 7th International Workshop.[23]
cCriteria include a normal annual basal and/or stimulated serum count, normal annual neck ultrasound, less aggressive medullary thyroid cancer family history, and family preference.

References

  1. 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.  [PUBMED Abstract]

  2. Kloos RT, Eng C, Evans DB, et al.: Medullary thyroid cancer: management guidelines of the American Thyroid Association. Thyroid 19 (6): 565-612, 2009.  [PUBMED Abstract]