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Cited by 848

The DOI matches its Crossref record; 8 of the 8 references it cites were found in a registry or an index.

Increased Risk of Cancer in the Peutz–Jeghers Syndrome

Giardiello, Francis M., Welsh, Stephen B., Hamilton, Stanley R., Offerhaus, G. Johan A., Gittelsohn, Alan M., Booker, Susan V., Krush, Anne J., Yardley, John H., Luk, Gordon D.·1987·New England Journal of Medicine

Abstract

The Peutz-Jeghers syndrome is an autosomal dominant hereditary disease characterized by hamartomatous polyps of the gastrointestinal tract and by mucocutaneous melanin deposits. The frequency of cancer in this syndrome has not been studied extensively. Therefore, we investigated 31 patients with the Peutz-Jeghers syndrome who were followed from 1973 to 1985. All cases of cancer were verified by histopathological review. Cancer developed in 15 of the 31 patients (48 percent)--gastrointestinal carcinomas in 4, nongastrointestinal carcinomas in 10, and multiple myeloma in 1. In addition, adenomatous polyps of the stomach and colon occurred in three other patients. The cancers were diagnosed when the patients were relatively young, but after the Peutz-Jeghers syndrome had been diagnosed (interval between diagnoses, 25 +/- 20 years; range, 1 to 64). According to relative-risk analysis, the observed development of cancer in the patients with the syndrome was 18 times greater than expected in the general population (P less than 0.0001). Our results suggest that patients with the Peutz-Jeghers syndrome have an increased risk for the development of cancer at gastrointestinal and nongastrointestinal sites.

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Metadata

Authors
Giardiello, F. M., Welsh, S. B., Hamilton, S. R., Offerhaus, G. J. A., Gittelsohn, A. M., Booker, S. V., Krush, A. J., Yardley, J. H., & Luk, G. D.
Venue
New England Journal of Medicine
Published
1987-06-11
Volume
316
Issue
24
Pages
1511-1514
ISSN
  • 0028-4793 (ISSN-L)
  • 1533-4406
OpenAlex
W2341084019
Cited by
848
References
8
FWCI
7.85
Type
journal article
Language
en
Open access
closed

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CoChat. (2026). Citation check: Increased Risk of Cancer in the Peutz–Jeghers Syndrome. https://app.cochat.ai/papers/10.1056/nejm198706113162404
The paper
Giardiello, F. M., Welsh, S. B., Hamilton, S. R., Offerhaus, G. J. A., Gittelsohn, A. M., Booker, S. V., Krush, A. J., Yardley, J. H., & Luk, G. D. (1987). Increased Risk of Cancer in the Peutz–Jeghers Syndrome. New England Journal of Medicine. https://doi.org/10.1056/nejm198706113162404

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

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