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Table of Contents
Vol. 19, No. 1, 2001
Issue release date: 2001
Section title: Paper
Dig Dis 2001;19:76–84
(DOI:10.1159/000050656)

Prevention of Pancreatic Cancer and Strategies for Management of Familial Pancreatic Cancer

Hruban R.H. · Canto M.I. · Yeo C.J.
Johns Hopkins Medical Institutions, Baltimore, Md., USA

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Article / Publication Details

First-Page Preview
Abstract of Paper

Published online: 5/18/2001

Number of Print Pages: 9
Number of Figures: 2
Number of Tables: 1

ISSN: 0257-2753 (Print)
eISSN: 1421-9875 (Online)

For additional information: http://www.karger.com/DDI

Abstract

At the current time, pancreatic cancer remains a difficult and typically fatal disease. A number of case reports and case-control epidemiologic studies have suggested that familial aggregation plays a role in as many as 10% of all pancreatic cancers. During the last several years, genetic alterations responsible for syndromes linked with pancreatic cancer have been identified. These genes include BRCA2, p16, PRSS1, STK11, and various mismatch repair genes. Unfortunately, most kindreds with a familial aggregation cannot be explained by one of these known genetic syndromes. Recent data from the National Familial Pancreas Tumor Registry at Johns Hopkins have estimated the prospective risk of pancreatic cancer among first-degree relatives of pancreatic cancer patients. The risk was estimated by comparing observed new cases of pancreatic cancer to expected numbers. In families where three first-degree relatives had been diagnosed with pancreatic cancer, the risk of another individual developing pancreatic cancer rose to a 57-fold increase over the basal risk. This article reviews the data concerning familial pancreatic cancer. Additionally, this article reviews the data concerning the histological precursors of invasive ductal adenocarcinoma of the pancreas: pancreatic intraepithelial neoplasias. Further, the current Johns Hopkins methodology used to screen for early pancreatic neoplasia in familial pancreatic cancer patients and in patients with familial Peutz-Jeghers syndrome is discussed. In summary, the notable advances in the field of molecular genetics have allowed for a better definition of the genetics of pancreatic cancer. With this knowledge has evolved a better understanding of several high-risk clinical syndromes associated with pancreatic cancer, familial pancreatic cancer, and the evolution of strategies to screen high-risk families for early pancreatic neoplasia.


Article / Publication Details

First-Page Preview
Abstract of Paper

Published online: 5/18/2001

Number of Print Pages: 9
Number of Figures: 2
Number of Tables: 1

ISSN: 0257-2753 (Print)
eISSN: 1421-9875 (Online)

For additional information: http://www.karger.com/DDI


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Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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