TY - JOUR
T1 - Very high risk of cancer in familial Peutz-Jeghers syndrome
AU - Giardiello, Francis M.
AU - Brensinger, Jill D.
AU - Tersmette, Anne C.
AU - Goodman, Steven N.
AU - Petersen, Gloria M.
AU - Booker, Susan V.
AU - Cruz-Correa, Marcia
AU - Offerhaus, Johan A.
PY - 2000
Y1 - 2000
N2 - Background & Aims: The Peutz-Jeghers syndrome (PJS) is an autosomal dominant polyposis disorder with increased risk of multiple cancers, but literature estimates of risk vary. Methods: We performed an individual patient meta-analysis to determine the relative risk (RR) of cancer in patients with PJS compared with the general population based on 210 individuals described in 6 publications. Results: For patients with PJS, the RR for all cancers was 15.2 (95% confidence limits [CL], 2, 19). A statistically significant increase of RR was noted for esophagus (57; CL, 2.5, 557), stomach (213; CL, 96, 368), small intestine (520; CL, 220, 1306), colon (84; CL, 47, 137), pancreas (132; CL, 44, 261), lung (17.0; CL, 5.4, 39), breast (15.2; CL, 7.6, 27), uterus (16.0; CL, 1.9, 56), ovary (27; CL, 7.3, 68), but not testicular or cervical malignancies. Cumulative risk for all cancer was 93% from age 15 to 64 years old. Conclusions: Patients with PJS are at very high relative and absolute risk for gastrointestinal and nongastrointestinal cancers.
AB - Background & Aims: The Peutz-Jeghers syndrome (PJS) is an autosomal dominant polyposis disorder with increased risk of multiple cancers, but literature estimates of risk vary. Methods: We performed an individual patient meta-analysis to determine the relative risk (RR) of cancer in patients with PJS compared with the general population based on 210 individuals described in 6 publications. Results: For patients with PJS, the RR for all cancers was 15.2 (95% confidence limits [CL], 2, 19). A statistically significant increase of RR was noted for esophagus (57; CL, 2.5, 557), stomach (213; CL, 96, 368), small intestine (520; CL, 220, 1306), colon (84; CL, 47, 137), pancreas (132; CL, 44, 261), lung (17.0; CL, 5.4, 39), breast (15.2; CL, 7.6, 27), uterus (16.0; CL, 1.9, 56), ovary (27; CL, 7.3, 68), but not testicular or cervical malignancies. Cumulative risk for all cancer was 93% from age 15 to 64 years old. Conclusions: Patients with PJS are at very high relative and absolute risk for gastrointestinal and nongastrointestinal cancers.
UR - http://www.scopus.com/inward/record.url?scp=0034464147&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0034464147&partnerID=8YFLogxK
U2 - 10.1053/gast.2000.20228
DO - 10.1053/gast.2000.20228
M3 - Article
C2 - 11113065
AN - SCOPUS:0034464147
SN - 0016-5085
VL - 119
SP - 1447
EP - 1453
JO - Gastroenterology
JF - Gastroenterology
IS - 6
ER -