Table 1

Baseline characteristics of the Dutch PJS cohort

Total144
Gender
 Male70 (49%)
 Female74 (51%)
Families61
Family history
 Familial PJS109 (76%)
 Sporadic24 (17%)
 Family history unknown11 (7%)
Fulfilling WHO criteria*142 (99%)
DNA mutation analysis92 (64%)
LKB1 mutation carrier 82/92 (89%)
Deceased48 (33%)
 Median age at death46 years (IQR 32–58 years)
Lost to follow-up 6 (4%)
Cancer48 (33%)
 Median age at diagnosis of first cancer46 years (IQR 35–55 years)
 2 primary cancers8
  • *Two patients not fulfilling the WHO criteria carry a proven LKB1 germline mutation.

  • PJS, Peutz-Jeghers syndrome.