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Clinical monitoring for pheochromocytoma and hyperparathyroidism in carriers of a mutation in the RET gene

Clinical monitoring for pheochromocytoma and hyperparathyroidism in carriers of a mutation in the RET gene
  Pheochromocytoma* Hyperparathyroidism
Risk RET codon mutation Recommended age to begin annual screening for pheochromocytoma Recommended age to begin annual screening for hyperparathyroidismΔ
Highest 918 11 years Not applicable
High 634, 883 11 years 11 years
Moderate 533, 609, 611, 618, 620, 630, 666, 768, 790, 804, 891, 912 16 years 16 years
MEN2: multiple endocrine neoplasia type 2; MTC: medullary thyroid cancer; CT: computed tomography; MRI: magnetic resonance imaging; PTH: parathyroid hormone.
* Patients with MEN2 and a diagnosis of MTC (regardless of age) must have pheochromocytoma excluded prior to thyroidectomy.
¶ Screening test: free plasma metanephrines or normetanephrines, or 24-hour urinary metanephrines and normetanephrines. If biochemical results positive, adrenal imaging with CT or MRI.
Δ Screening test: serum calcium and, if elevated, PTH.
Data from: Wells SA Jr, Asa SL, Dralle H, et al. Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma. Thyroid 2015; 25:567.
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