Increased risk for cancer in patients with the Peutz-Jeghers syndrome.
BACKGROUND
Some reports describe an increased risk for cancer in patients with the Peutz-Jeghers syndrome.


OBJECTIVE
To characterize occurrences of cancer in a large cohort of patients with the Peutz-Jeghers syndrome.


METHODS
Retrospective cohort study.


METHODS
Tertiary care center.


METHODS
34 patients with the Peutz-Jeghers syndrome identified from Mayo Clinic records from 1945 to 1996.


METHODS
Cases of cancer documented by chart review and telephone follow-up.


RESULTS
26 cases of noncutaneous cancer developed in 18 of the 34 patients: 10 cases of gastrointestinal cancer and 16 cases of extraintestinal cancer. With the use of SEER (Surveillance, Epidemiology, and End Results) data for comparison, the relative risk for cancer was 18.5 (95% CI, 8.5 to 35.2) in women with the Peutz-Jeghers syndrome and 6.2 (CI, 2.5 to 12.8) in men with the syndrome (P = 0.001). In women, the relative risk for breast and gynecologic cancer was 20.3 (CI, 7.4 to 44.2).


CONCLUSIONS
The Peutz-Jeghers syndrome is associated with an increased risk for cancer. The relative risk for breast and gynecologic cancers is particularly high.
Boardman
L A
LA
Mayo Clinic, Rochester, Minnesota 55905, USA.
Thibodeau
S N
SN
Schaid
D J
DJ
Lindor
N M
NM
McDonnell
S K
SK
Burgart
L J
LJ
Ahlquist
D A
DA
Podratz
K C
KC
Pittelkow
M
M
Hartmann
L C
LC
D000293:Adolescent; D000328:Adult; D001943:Breast Neoplasms / Q000453:epidemiology / Q000209:etiology; D015331:Cohort Studies; D004198:Disease Susceptibility; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000453:epidemiology / Q000209:etiology; D005833:Genital Neoplasms, Female / Q000453:epidemiology / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000453:epidemiology / Q000209:etiology*; D010580:Peutz-Jeghers Syndrome / Q000150:complications*; D012189:Retrospective Studies; D012307:Risk Factors; D018426:SEER Program; D012737:Sex Factors; D014481:United States / Q000453:epidemiology