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Familial risk factors for pancreatic cancer and screening of high-risk patients

Author
Marcia Irene Canto, MD, MHS
Section Editor
J Thomas Lamont, MD
Deputy Editors
Diane MF Savarese, MD
Shilpa Grover, MD, MPH

INTRODUCTION

Pancreatic ductal adenocarcinoma (pancreatic cancer, PC) is one of the leading causes of cancer-related mortality worldwide [1]. In the United States, PC ranks fourth in leading causes of cancer deaths among men and women [2]. Most symptomatic patients with PC have advanced, incurable disease at diagnosis. Even in those with apparently resectable tumors, prognosis is poor. Given that outcomes may be better following resection of small invasive cancers, it is hoped that screening and detection of asymptomatic, early, potentially curable PC and its precursors will improve outcomes. Individuals at increased risk for PC based upon family history or an identifiable genetic predisposition are potential targets for selective screening and curative or preventive treatment.

This topic reviews the epidemiology and genetic basis of familial PC and familial PC-associated genetic syndromes, the diagnostic tests used for screening, and the risks and benefits of screening for PC. A more detailed discussion of epidemiology and risk factors for PC, and the clinical evaluation and staging workup of newly diagnosed PC, are addressed separately. (See "Epidemiology and nonfamilial risk factors for exocrine pancreatic cancer" and "Clinical manifestations, diagnosis, and staging of exocrine pancreatic cancer".)

EPIDEMIOLOGY AND RISK FACTORS

An estimated 10 to 15 percent of PCs are attributable to genetic causes [3-7]. PC aggregates in some families, and approximately 5 to 10 percent of individuals with PC have a family history of the disease [6,8].

There are two broad categories of hereditary risk for PC:

Genetic predisposition syndromes associated with PC (table 1)

                                    

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Literature review current through: Nov 2016. | This topic last updated: Tue Sep 20 00:00:00 GMT+00:00 2016.
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