Table 3.
Risk stratification and recommendations for prophylactic thyroidectomy in children with RET mutations
| MTC risk level | RET mutation | Exon | Age for prophylactic surgery in children |
|---|---|---|---|
| Highest | M918T1 | 16 | First year or the first months of life |
| High | C634F/G/R/S/W/Y | 11 | At or before 5 years of age based on serum CT |
| A883F1 | 15 | levels | |
| Moderate | G553C | 8 | To be performed when the serum CT level becomes elevated, or in childhood if parents do not wish to embark on a lengthy period of periodic evaluation |
| C609F/G/R/S/Y | 10 | ||
| C611F/G/S/Y/W | |||
| C618F/R/S, C620F/R/S | |||
| C630R/Y, D631Y, K666E | 11 | ||
| E768D, L790F | 13 | ||
| V804L/M | 14 | ||
| S891A, R912P | 16 | ||
Adapted from Wells et al. [68]. MTC, medullary thyroid carcinoma; RET, rearranged during transfection; CT, calcitonin.
1
Mutations associated with multiple endocrine neoplasia type 2B.