Very high risk of cancer in familial Peutz-Jeghers syndrome

Francis M. Giardiello, Jill D. Brensinger, Anne C. Tersmette, Steven N. Goodman, Gloria M Petersen, Susan V. Booker, Marcia Cruz-Correa, Johan A. Offerhaus

Research output: Contribution to journalArticle

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Abstract

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.

Original languageEnglish (US)
Pages (from-to)1447-1453
Number of pages7
JournalGastroenterology
Volume119
Issue number6
StatePublished - 2000
Externally publishedYes

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Peutz-Jeghers Syndrome
Neoplasms
Gastrointestinal Neoplasms
Esophagus
Small Intestine
Uterus
Publications
Meta-Analysis
Pancreas
Ovary
Stomach
Colon
Breast
Lung

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Giardiello, F. M., Brensinger, J. D., Tersmette, A. C., Goodman, S. N., Petersen, G. M., Booker, S. V., ... Offerhaus, J. A. (2000). Very high risk of cancer in familial Peutz-Jeghers syndrome. Gastroenterology, 119(6), 1447-1453.

Very high risk of cancer in familial Peutz-Jeghers syndrome. / Giardiello, Francis M.; Brensinger, Jill D.; Tersmette, Anne C.; Goodman, Steven N.; Petersen, Gloria M; Booker, Susan V.; Cruz-Correa, Marcia; Offerhaus, Johan A.

In: Gastroenterology, Vol. 119, No. 6, 2000, p. 1447-1453.

Research output: Contribution to journalArticle

Giardiello, FM, Brensinger, JD, Tersmette, AC, Goodman, SN, Petersen, GM, Booker, SV, Cruz-Correa, M & Offerhaus, JA 2000, 'Very high risk of cancer in familial Peutz-Jeghers syndrome', Gastroenterology, vol. 119, no. 6, pp. 1447-1453.
Giardiello FM, Brensinger JD, Tersmette AC, Goodman SN, Petersen GM, Booker SV et al. Very high risk of cancer in familial Peutz-Jeghers syndrome. Gastroenterology. 2000;119(6):1447-1453.
Giardiello, Francis M. ; Brensinger, Jill D. ; Tersmette, Anne C. ; Goodman, Steven N. ; Petersen, Gloria M ; Booker, Susan V. ; Cruz-Correa, Marcia ; Offerhaus, Johan A. / Very high risk of cancer in familial Peutz-Jeghers syndrome. In: Gastroenterology. 2000 ; Vol. 119, No. 6. pp. 1447-1453.
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AU - Giardiello, Francis M.

AU - Brensinger, Jill D.

AU - Tersmette, Anne C.

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AU - Petersen, Gloria M

AU - Booker, Susan V.

AU - Cruz-Correa, Marcia

AU - Offerhaus, Johan A.

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