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 |
| D | 883, 918, and compound heterozygotes V804M+E805K, V804M+Y806C, and V804M+S904C | ASAP and within the first y of life | ASAP and within the first y of life. |
| C | 634 | <3–5 y | Before age 5 y. |
| B | 609b, 611, 618, 620, 630b, and compound heterozygote V804M+V778I | <3–5 y | Consider surgery before age 5 y. May delay surgery after age 5 y if criteria are met.c |
| A | 768, 790, 791, 804, 891 | <3–5 y | May 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
- 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]
- 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]
