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Table 2 Management of MEN 2 and FMTC patients according to RET genotypea

From: An Introduction to Managing Medullary Thyroid Cancer

       Recommended age for surgery   
risk category risk level RET codon youngest age at first diagnosis of MTC youngest age at first diagnosis of PCC youngest age at first diagnosis of HPT thyroidectomy central lymph node dissection recommended age to start screening for PCC recommended age to start screening for HPT
highest 3 883 not described not described - <1 years <1 years 10 years -
highest 3 918 9 months 12 years - <1 years <1 years 10 years -
high 2 609 5 years 22 years unspecified <5 years ≥20 yearsb 20 years 20 years
high 2 611 7 years 30 years unspecified <5 years ≥20 yearsb 20 years 20 years
high 2 618 7 years 29 years 41 years <5 years ≥20 yearsb 20 years 20 years
high 2 620 6 years 22 years unspecified <5 years ≥20 yearsb 20 years 20 years
high 2 630 12 months - 32 years <2 years ≥10 yearsb 5 years 20 years
high 2 634 13 months 5 years 10 years <2 years ≥10 yearsb 5 years <10 years
high 2 912 14 years - - <5 years ≥10 yearsb 20 years 20 years
least high 1 533 21 years - - 5-10 years ≥20 yearsb 20 years 20 years
least high 1 649 44 years - - 5-10 years ≥20 yearsb 20 years 20 years
least high 1 666 35 years 35 years - 5-10 years ≥20 yearsb 20 years 20 years
least high 1 768 22 years 59 years - 5-10 years ≥20 yearsb 20 years 20 years
least high 1 790 10 years 28 years - 5-10 years ≥20 yearsb 20 years 20 years
least high 1 791 21 years 38 years 38 years 5-10 years ≥20 yearsb 20 years 20 years
least high 1 804 6 years 28 years 10 years 5-10 years ≥20 yearsb 20 years <10 years
least high 1 891 13 years 52 years - 5-10 years ≥20 yearsb 20 years 20 years
  1. athe recommendations are made based on results from recent literature [35]. This table was adapted from De Groot et al. [35]. Copyright 2006, The Endocrine Society.
  2. bno consensus has been reached for the extent of surgery for MTC in patients carrying these germline mutations in the RET gene. Recommendations are based on recent literature. If basal or pentagastrin-stimulated calcitonin levels are abnormal in RET mutation carriers, thyroidectomy and central lymph node dissection should be performed immediately.
  3. For mutations at codons 600, 603, 606, 778, 781, and 852 insufficient data are available for recommendations, but most likely they belong to risk level 1.
  4. MEN 2, multiple endocrine neoplasia type 2; FMTC, familial medullary thyroid cancer; RET, rearranged during transfection; MTC, medullary thyroid cancer; PCC, pheochromocytoma; HPT, hyperparathyroidism.