N=133 | |
Gender | |
Male | 64 (48%) |
Female | 69 (52%) |
Age at the end of follow-up | |
Median age (range) patients alive (n=89) | 34 (4–75) years |
Median age (range) patients deceased (n=42) | 45 (3–76) years |
Patients lost to follow-up (n=2) | NA |
Family history | |
Familial PJS | 100 (75%) |
Sporadic PJS | 23 (17%) |
Family history unknown | 10 (8%) |
DNA mutation analysis | 80 (60%) |
STK11 mutation carrier | 77 / 80 (96%) |
Cancer | 42 (32%) |