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Multiple Endocrine Neoplasia Type 2

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

    A study of young, clinically asymptomatic individuals with MEN2A sought to determine if early thyroidectomy could prevent or cure MTC.[194] This study included 50 consecutively identified RET mutation carriers who underwent thyroidectomy at 19 years or younger. Preoperative screening for CCH included basal and stimulated calcitonin levels and postoperative follow-up consisted of annual physical exam and intermittent basal and stimulated calcitonin measurements. All 50 individuals had at least 5 years of follow-up. Although MTC was identified in 33 of 50 patients at the time of surgery, in 44 of 50 (88%) there was no evidence of persistent or recurrent disease at a mean of 7 years follow-up. Six patients had basal or stimulated calcitonin abnormalities thought to represent residual MTC. None of the 22 patients operated on prior to age 8 years had any evidence of MTC. The data suggested that there was a lower incidence of persistent or recurrent disease in patients who had thyroidectomy earlier in life (defined as younger than 8 years) and who had no evidence of lymph node metastases.

    Normal preoperative basal calcitonin does not exclude the possibility of the patient having MTC. In one study of 80 RET mutation carriers, 14 carriers had normal calcitonin tests and eight of these patients had small foci of MTC discovered at thyroidectomy.[13] Another study confirmed these findings,[81] as 14 children had total thyroidectomy based on positive genetic testing for MEN2; MTC was present in 11 and only four had elevated stimulated calcitonin levels prior to surgery. Although basal calcitonin levels may not be able to identify all patients with MTC preoperatively, this test has utility as a predictor of postoperative remission, lymph node metastases, and distant metastases.[195] In one study of 224 patients from a single institution, preoperative basal calcitonin levels greater than 500 pg/mL predicted failure to achieve biochemical remission.[195] The authors of this study found that nodal metastases started appearing at basal calcitonin levels of 40 pg/mL (normal, <10 pg/mL). In node-positive patients, distant metastases emerged at basal calcitonin levels of 150 pg/mL to 400 pg/mL. Using current sensitive calcitonin assays, a study of 308 RET carriers found that a normal basal preoperative calcitonin excluded the presence of lymph node metastases (100% negative predictive value).[196] Therefore, the preoperative basal calcitonin level is a useful prognostic indicator and may help guide the surgical approach.

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