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Published in: Hereditary Cancer in Clinical Practice 1/2017

Open Access 01-12-2017 | Review

Hereditary pancreatic cancer: related syndromes and clinical perspective

Authors: Sergio Carrera, Aintzane Sancho, Eider Azkona, Josune Azkuna, Guillermo Lopez-Vivanco

Published in: Hereditary Cancer in Clinical Practice | Issue 1/2017

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Abstract

Pancreatic cancer is a very aggressive disease with a poor prognosis. The majority of them are attributed to sporadic causes, especially to many modifiable risk factors such as tobacco or alcohol abuse. The principal histologic subtype of pancreatic cancer is ductal adenocarcinoma. Pancreatic neuroendocrine tumors, which constitute a more indolent entity, represent second type of pancreatic cancer in terms of incidence. Individuals with a family history of pancreatic cancer carry an increased risk of developing the disease, which may be related to an underlying hereditary component. Unfortunately, in the majority of these families the suspected germline genetic cause responsible of the disease will not be identified, but approximately in a 20% of the cases a hereditary cancer predisposition syndrome with increased risk of pancreatic cancer development can be recognized. This review will be focused on the leading hereditary cancer syndromes related to pancreatic ductal adenocarcinoma and pancreatic neuroendocrine tumors. Additionally, we will try to explain clinical aspects related to the identification of germline mutations in pancreatic cancer patients and their potential implications in oncologic treatment decisions.
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Metadata
Title
Hereditary pancreatic cancer: related syndromes and clinical perspective
Authors
Sergio Carrera
Aintzane Sancho
Eider Azkona
Josune Azkuna
Guillermo Lopez-Vivanco
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Hereditary Cancer in Clinical Practice / Issue 1/2017
Electronic ISSN: 1897-4287
DOI
https://doi.org/10.1186/s13053-017-0069-6

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