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Multiple Endocrine Neoplasia Type 2

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

    Level of evidence: 5

    Screening of at-risk individuals for hyperparathyroidism

    MEN2-related hyperparathyroidism is generally associated with mild, often asymptomatic hypercalcemia early in the natural history of the disease, which, if left untreated, may become symptomatic.[70] Childhood hyperparathyroidism is rare in MEN2. Three studies found the median age at diagnosis was about 38 years.[70,203,204] The ATA provides recommendations for annual screening for hyperparathyroidism.[25] Annual screening should begin at age 8 years in carriers of mutations in codons 630 and 634 and at age 20 years for carriers of other MEN2A RET mutations. Patients with mutations associated only with FMTC should have periodic testing after the age of 20 years. Testing should include albumin-corrected calcium or ionized serum calcium with or without intact PTH measurement.

    Level of evidence: 5

    Screening of at-risk individuals in kindreds without an identifiableRETmutation

    MEN2A: Risk-reducing thyroidectomy is not routinely offered to at-risk individuals if the disorder is unconfirmed. The screening protocol for MTC is an annual calcitonin stimulation test; however, caution must be used in interpreting test results because CCH that is not a precursor to MTC occurs in about 5% of the population.[12,13,205] In addition, there is significant risk of false-negative test results in patients younger than 15 years.[13] Screening for pheochromocytoma and parathyroid disease is the same as described above.

    FMTC: Annual screening for MTC, as for MEN2A.

    Level of evidence: 5

    Treatment for those with MTC

    Standard treatment for adults with MTC is surgical removal of the entire thyroid gland, including the posterior capsule, and central lymph node dissection. Children with MEN2B having prophylactic thyroidectomy within the first year of life may not require central neck dissection unless there is radiological evidence of nodal disease.[25] Likewise, children with MEN2A or FMTC having prophylactic thyroidectomy before 3 to 5 years of age should not have a central neck dissection in the absence of radiological evidence of metastatic lymph node involvement. The ATA also recommends that MEN2A and FMTC patients older than 5 years or asymptomatic MEN2B patients older than 1 year have a preoperative basal calcitonin test and neck ultrasound. A basal calcitonin level over 40 pg/mL or thyroid nodules greater than or equal to 5 mm requires further evaluation, as the patient may have more extensive disease requiring nodal dissection. If an MEN2B patient older than 1 year has nodules smaller than 5 mm or basal calcitonin lower than 40 pg/mL, then total thyroidectomy may be sufficient therapy, but the ATA task force favors prophylactic central neck dissection without lateral compartment dissection in the absence of radiographic evidence of metastatic involvement (level C recommendation).[25] See Table 6 for complete details.

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