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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.