Table 1 Characteristics of MEN-1 pancreatic neuroendocrine lesions

From: Loss of ATRX or DAXX expression and concomitant acquisition of the alternative lengthening of telomeres phenotype are late events in a small subset of MEN-1 syndrome pancreatic neuroendocrine tumors

 

Microadenomas

Pancreatic neuroendocrine tumors

Lymph node metastases

Total (n=109)

47

50

12

Mitoses (per 10 high power fields)

 <2 (WHO grade 1)

46

43

12

 2–20 (WHO grade 2)

1

7

0

 >20 (WHO grade 3)

0

0

0

KI67 proliferation index (%)

 <2 (WHO grade 1)

41a

39

7a

 3–20 (WHO grade 2)

1

11

4

 >20 (WHO grade 3)

0

0

0

ATRX nuclear labeling

 Positive

47

49

12

 Negative

0

0

0

 Heterogeneous

0

1b

0

DAXX nuclear labeling

 Positive

47

47

10

 Negative

0

2

2

 Heterogeneous

0

1

0

Alternative lengthening of telomeres phenotype

 Negative

47

47

10

 Positive

0

3

2

  1. Abbreviations: ATRX, alpha thalassemia/mental retardation X-linked; DAXX, death domain-associated protein; MEN-1, multiple endocrine neoplasia-1; WHO, World Health Organization.21
  2. aKI67 immunolabeling was available for 42 of 47 microadenomas and 11 of 12 lymph nodes.
  3. bThis tumor also showed loss of DAXX.