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Hereditary pancreatic cancer: related syndromes and clinical perspective

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 neuroe...

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Autores principales: Carrera, Sergio, Sancho, Aintzane, Azkona, Eider, Azkuna, Josune, Lopez-Vivanco, Guillermo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5490219/
https://www.ncbi.nlm.nih.gov/pubmed/28670351
http://dx.doi.org/10.1186/s13053-017-0069-6
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author Carrera, Sergio
Sancho, Aintzane
Azkona, Eider
Azkuna, Josune
Lopez-Vivanco, Guillermo
author_facet Carrera, Sergio
Sancho, Aintzane
Azkona, Eider
Azkuna, Josune
Lopez-Vivanco, Guillermo
author_sort Carrera, Sergio
collection PubMed
description 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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spelling pubmed-54902192017-06-30 Hereditary pancreatic cancer: related syndromes and clinical perspective Carrera, Sergio Sancho, Aintzane Azkona, Eider Azkuna, Josune Lopez-Vivanco, Guillermo Hered Cancer Clin Pract Review 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. BioMed Central 2017-06-28 /pmc/articles/PMC5490219/ /pubmed/28670351 http://dx.doi.org/10.1186/s13053-017-0069-6 Text en © The Author(s). 2017 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Review
Carrera, Sergio
Sancho, Aintzane
Azkona, Eider
Azkuna, Josune
Lopez-Vivanco, Guillermo
Hereditary pancreatic cancer: related syndromes and clinical perspective
title Hereditary pancreatic cancer: related syndromes and clinical perspective
title_full Hereditary pancreatic cancer: related syndromes and clinical perspective
title_fullStr Hereditary pancreatic cancer: related syndromes and clinical perspective
title_full_unstemmed Hereditary pancreatic cancer: related syndromes and clinical perspective
title_short Hereditary pancreatic cancer: related syndromes and clinical perspective
title_sort hereditary pancreatic cancer: related syndromes and clinical perspective
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5490219/
https://www.ncbi.nlm.nih.gov/pubmed/28670351
http://dx.doi.org/10.1186/s13053-017-0069-6
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