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Table 1 Mutation, TNM, surgery, Ki-67 (%) of the largest tumor, and follow-up of 6 MEN-1 patients

From: Intertumor heterogeneity in 60 pancreatic neuroendocrine tumors associated with multiple endocrine neoplasia type 1

Patient

Mutation

Gender

Age

T

N

M

Ki-67%

function

Surgery

Follow-up

Status

Years

ENETS

UICC

A

Exon 2, del 4 bp (c.247_250delCTGT);

- > Termination after amino acid 116

m

29

3

2

0

0

2

F (WDHA-Syndrome)

TP

DF

12

B

Intron 4, G > A -9 bp

- > Alternative splicing

f

60

2

2

1

0

8

NF

TP

PD (M?)

10

C

Exon 9, Q405X, CAG > TAG (Gln > Stop)

- > Termination after amino acid 404

m

60

2

2

0

0

2

NF

TP

DF

7

D

Exon 3, Codon 179 GAG>AAG (Glu > Lys)

- > AS Exchange

m

15

2

1

0

0

3

F (Hyper-insulinism)

DP

DF

29

E

Exon 4, p.I247N, ATT > AAT (Ile > Asn)

WORLDWIDE INDEX-CASE

f

38

2

2

0

0

1

NF

TP

DF [+]

4

F

Exon 3, del4bp (amino acid 210/211)

- > Termination after amino acid 209

m

33

3

2

1

0

1

F (subclinical Hyper-insulinism)

DP, E

PD (N)

21

  1. T tumor classification of the largest tumor, N lymph node, Ki-67 Index in %, M distant metastasis, m male, f female
  2. F functioning, NF non-functioning
  3. TP fotal pancreatectomy, DP distal pancreatic resection, E enucleation
  4. DF disease free, PD pogressive disease; [+]: died unrelated to MEN-1