Table 1.

Cumulative cancer risk by site and age in Peutz-Jeghers syndrome patients

Type of cancerCancer risk by age (95% confidence intervals)
20 y30 y40 y50 y60 y70 y
All cancers (ICD 140-208)2 (0.8-4) [0.3]5 (3-8) [0.6]17 (13-23) [1.4]31 (24-39) [3.4]60 (50-71) [8.2]85 (68-96) [17.7]
Gastrointestinal (ICD 150-159)— [<0.1]1 (0.4-3) [<0.1]9 (5-14) [0.1]15 (10-22) [0.3]33 (23-45) [1.3]57 (39-76) [3.9]
Breast (female; ICD 174)— [<0.1]— [<0.1]8 (4-17) [0.5]13 (7-24) [1.8]31 (18-50) [4.3]45 (27-68) [7.0]
Gynecologic (ICD 179-184)— [<0.1]1 (0.4-6) [0.1]3 (0.9-9) [0.4]8 (4-19) [0.7]18 (9-34) [1.3]18 (9-34) [2.1]
Pancreas (ICD 157)— [<0.1]— [<0.1]3 (1-7) [<0.1]5 (2-10) [<0.1]7 (3-16) [0.2]11 (5-24) [0.5]
Lung (ICD C34)
— [<0.1]— [<0.1]1 (0.1-6) [<0.1]4 (1-11) [0.2]13 (6-28) [1.3]17 (8-36) [4.7]
[<0.1][<0.1]1 (0.1-6) [<0.1]— [0.1]— [0.6]— [2.0]
  • NOTE: Population risk shown in square brackets are risks for males and females provided separately; similarly, lung cancer risks in male and female Peutz-Jeghers syndrome patients are provided separately.