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Medullary Thyroid Carcinoma

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8 Treatment Î Serum thyroid stimulating hormone (TSH) should be measured within 4-6 weeks postoperatively. Replacement therapy with levothyroxine should be administered with the goal of maintaining serum TSH levels in the euthyroid range. (B) Î Serum calcium levels should be monitored postoperatively. Oral calcium and vitamin D should be administered to patients who develop symptomatic hypocalcemia. Chronic replacement therapy is indicated in patients who cannot be weaned from the initial medication. (B). Î Experienced physicians and surgeons in tertiary care centers should be responsible for the management of children with MEN2A or MEN2B. (B) Î Children in the ATA-HST category with a RET codon M918T mutation should have a thyroidectomy in the first year of life, perhaps even in the first months of life. In the absence of suspicious lymph nodes the performance of a central neck dissection should be based on whether the parathyroid glands can be identified and left in situ or autotransplanted. The surgeon and pediatrician caring for the patient, in consultation with the child's parents, should decide the timing of thyroidectomy. (C) Î Children in the ATA-H category should have a thyroidectomy performed at age 5 years or earlier, based on the detection of elevated serum Ctn levels. A central neck dissection should be performed in children with serum Ctn levels >40 pg/mL, or with evidence on imaging or direct observation of lymph node metastases. The surgeon and pediatrician caring for the patient, in consultation with the child's parents, should decide the timing of thyroidectomy. (B) Î Children in the ATA-MOD category should have a physical examination, US of the neck, and measurement of serum Ctn levels beginning around 5 years of age. The timing of thyroidectomy should be based on the detection of an elevated serum Ctn level. However, 6-month or annual evaluations may extend to several years or decades. (B) Note: Parents who are concerned about a long-term evaluation program, may opt to have their child's thyroid gland removed around 5 years of age. The surgeon and pediatrician caring for the patient, in consultation with the child's parents, should decide the timing of thyroidectomy. Î Screening for PHEO should begin by age 11 years for children in the ATA-H and ATA-HST categories and by age 16 years in children in the ATA-MOD category. Screening consists of measuring free plasma metanephrines and normetanephrines, or 24-hour urinary metanephrines and normetanephrines. Adrenal imaging with CT or MRI is indicated in patients with positive biochemical results. (C)

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