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Table 1 Estimated cancer risk

From: Danish guidelines for management of non-APC-associated hereditary polyposis syndromes

Syndrome

Site of cancer

Cumulative lifetime risk or frequency among carriers

Age of debut

Peutz-Jeghers Syndrome [2]

Colon/rectum

39%

42-46y

Stomach

29%

30-40y

Small bowel

13%

37-42y

Breast

32–54%

37-59y

Ovarian

21%

28y

Cervix (adenoma malignum)

10%

34-40y

Uterus

9%

43y

Pancreas

11–38%

41–52y

Testicular (sertoli cell tumour)

9%

6-9y

Lung

7–17%

47y

Juvenile Polyposis Syndrome

Colon/rectum

38% [3]

36.0 (48) (median)

Gastric

21% [2]

44.0 (48)

POLE-associated polyposis [4]

Colon/rectum

28% (M), 21%(F) For p.Leu424VAL: 97% (M), 92% (F)

50.2 (49) (mean)

Uterus

?

 

Ovaries

?

 

Pancreas

?

 

Malignant melanoma

?

 

POLD1-associated polyposis [4]

Colon/rectum

90% (M), 82% (F)

39.7 (49) (median)

Uterus

?

?

Breast cancer

?

?

Ovarian

?

?

Lymphoma

?

?

Bladder

?

?

AXIN2-associated polyposis

Colon/rectum

13/35 individuals

36–80+

MUTYH- associated polyposis [5]

Colon/rectum

80–90%

48.0 (median)

Duodenum

4%

61.0

Ovaries

6–14%

51.0

Bladder

6–8% (females), 6–25% males)

61.0

Melanoma

?

?

Breast

?

?

Uterus

3%

51.0

NTHL1-associated polyposis [6]

Colon/rectum

16/29 individuals

61.0 median, (33-73y)

Breast

9/15 female individuals

48.5 (38-63y)

Uterus

5/15 Uterus (precancerous and cancerous)

57.0 (6–74 y).

Duodenum

?

 

CMMRD [7]

Colon/rectum

59/146 individuals

8-48y

Duodenum

18/149 individuals

11-42y

Hematologic malignancies

?

¿

brain tumors

 

¿

GREM1-associated mixed polyposis

CRC

?

?

Serrated Polyposis Syndrome [8]

CRC

15–35%.

53.9 (median)