From: Unusual Cancers of Childhood Treatment (PDQ®)
PDQ Cancer Information Summaries [Internet].
Bethesda (MD): National Cancer Institute (US); 2002-.
NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.
Table 2. Risk Levels and Management Based on Common RET Mutations Detected on Genetic Screeninga
| Medullary Thyroid Carcinoma Risk Level | |||
|---|---|---|---|
| Highest (MEN2B) | High (MEN2A) | Moderate (MEN2A) | |
| RET Mutation | M918T | A883F, C634F/G/R/S/W/Y | G533C, C609F/G/R/S/Y, C611F/G/S/Y/W, C618F/R/S, C620F/R/S, C630R/Y, D631Y, K666E, E768D, L790F, V804L, V804M, S891A, R912P |
| Age for Prophylactic Thyroidectomy | Total thyroidectomy in the first year of life, ideally in the first months of life. | Total thyroidectomy at or before age 5 y based on serum calcitonin levels. | Total thyroidectomy to be performed when the serum calcitonin level is above the normal range or at convenience if the parents do not wish to embark on a lengthy period of surveillance. |
MEN2A = multiple endocrine neoplasia type 2A; MEN2B = multiple endocrine neoplasia type 2B.
aAdapted from Wells et al.[95]