A 50-year-old woman is found to have MEN2A syndrome. Genetic testing reveals a RET proto-oncogene mutation at codon 634. Which prophylactic surgery schedule is recommended per ATA guidelines?

• A Prophylactic thyroidectomy within 6 months of age
• B Prophylactic thyroidectomy by age 5 years (moderate-risk category) ✓
• C Prophylactic thyroidectomy only when calcitonin becomes elevated
• D No surgery; screen with annual calcitonin levels

Explanation

ATA guidelines stratify RET mutations into highest-risk (codons 918, 883 — thyroidectomy within 6 months of age), high-risk (codons 634, 618, 620 — thyroidectomy within the first 6 months to 5 years), and moderate-risk. Codon 634 mutations carry high risk of early medullary thyroid carcinoma, so prophylactic thyroidectomy by age 5 (or earlier if calcitonin rises) is recommended. Waiting for calcitonin elevation may mean the cancer has already developed.

Reference: Bailey & Love's Short Practice of Surgery, 27th ed.

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