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Multiple Endocrine Neoplasia Type 2

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

    Risk levelMutated Codon(s)Age ofRETTestingTiming of Prophylactic Thyroidectomy
    a Adapted from Kloos et al.[25]
    b These mutations had not been reported at the time of the 7th International Workshop.[23]
    c Criteria include a normal annual basal and/or stimulated serum count, normal annual neck ultrasound, less aggressive medullary thyroid cancer family history, and family preference.
    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

    In a study of biochemical screening in a large family with MEN2A performed before mutation analysis became available, 22 family members without evidence of clinical disease had elevated calcitonin and underwent thyroidectomy. During a mean follow-up period of 11 years, all remained free of clinical disease, and 3 out of 22 had transient elevation of postoperative calcitonin levels.[9] The use of biochemical screening is limited, however, by the lack of data on age-specific calcitonin levels in children under 3 years of age; caution should be used when interpreting these values in this age group.[25]

    A study of 93 patients with MEN2 from a Dutch tumor registry documented the importance of early prophylactic thyroidectomy.[190] This group of patients represented all known Dutch patients with hereditary MTC; the majority of cases (67%) were codon 634 mutations; only 6% were MEN2B cases. Patients in this series were screened with either biochemical testing (pre-RET era) or RET mutation analysis. In both groups, patients who underwent surgery at a later age than recommended by current guidelines (see Table 5), but the percentage from the pre-RET era was significantly higher (96% vs. 69%, P = .004). Older age at prophylactic thyroidectomy was significantly associated with a higher risk of postoperative persistent/recurrent disease. Although there is concern that young age at total thyroidectomy is associated with higher risk of surgical complications, this study found no such evidence.

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