Very high risk of cancer in familial Peutz-Jeghers syndrome

F. M. Giardiello; J. D. Brensinger; A. C. Tersmette; S. N. Goodman; G. M. Petersen; S. V. Booker; M. Cruz-Correa; J. A. Offerhaus

doi:https://doi.org/10.1053/gast.2000.20228

Very high risk of cancer in familial Peutz-Jeghers syndrome

F. M. Giardiello, J. D. Brensinger, A. C. Tersmette, S. N. Goodman, G. M. Petersen, S. V. Booker, M. Cruz-Correa, J. A. Offerhaus

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Research output: Contribution to journal › Article* › Academic › peer-review

Abstract

The Peutz-Jeghers syndrome (PJS) is an autosomal dominant polyposis disorder with increased risk of multiple cancers, but literature estimates of risk vary. 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. 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. Patients with PJS are at very high relative and absolute risk for gastrointestinal and nongastrointestinal cancers

Original language	English
Pages (from-to)	1447-1453
Journal	Gastroenterology
Volume	119
Issue number	6
DOIs	https://doi.org/10.1053/gast.2000.20228
Publication status	Published - 2000

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https://doi.org/10.1053/gast.2000.20228

Cite this

@article{3a03cf2940054798babda252931f4e54,

title = "Very high risk of cancer in familial Peutz-Jeghers syndrome",

abstract = "The Peutz-Jeghers syndrome (PJS) is an autosomal dominant polyposis disorder with increased risk of multiple cancers, but literature estimates of risk vary. 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. 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. Patients with PJS are at very high relative and absolute risk for gastrointestinal and nongastrointestinal cancers",

author = "Giardiello, {F. M.} and Brensinger, {J. D.} and Tersmette, {A. C.} and Goodman, {S. N.} and Petersen, {G. M.} and Booker, {S. V.} and M. Cruz-Correa and Offerhaus, {J. A.}",

year = "2000",

doi = "https://doi.org/10.1053/gast.2000.20228",

language = "English",

volume = "119",

pages = "1447--1453",

journal = "Gastroenterology",

issn = "0016-5085",

publisher = "W.B. Saunders Ltd",

number = "6",

}

TY - JOUR

T1 - Very high risk of cancer in familial Peutz-Jeghers syndrome

AU - Giardiello, F. M.

AU - Brensinger, J. D.

AU - Tersmette, A. C.

AU - Goodman, S. N.

AU - Petersen, G. M.

AU - Booker, S. V.

AU - Cruz-Correa, M.

AU - Offerhaus, J. A.

PY - 2000

Y1 - 2000

N2 - The Peutz-Jeghers syndrome (PJS) is an autosomal dominant polyposis disorder with increased risk of multiple cancers, but literature estimates of risk vary. 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. 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. Patients with PJS are at very high relative and absolute risk for gastrointestinal and nongastrointestinal cancers

AB - The Peutz-Jeghers syndrome (PJS) is an autosomal dominant polyposis disorder with increased risk of multiple cancers, but literature estimates of risk vary. 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. 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. Patients with PJS are at very high relative and absolute risk for gastrointestinal and nongastrointestinal cancers

U2 - https://doi.org/10.1053/gast.2000.20228

DO - https://doi.org/10.1053/gast.2000.20228

M3 - Article*

C2 - 11113065

VL - 119

SP - 1447

EP - 1453

JO - Gastroenterology

JF - Gastroenterology

SN - 0016-5085

IS - 6

ER -