Hereditary pancreatic cancer: related syndromes and clinical perspective
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. Pancreat...
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doaj-cac1ff545284492891dded6d9a8607252020-11-24T20:49:02ZengBMCHereditary Cancer in Clinical Practice1897-42872017-06-011511910.1186/s13053-017-0069-6Hereditary pancreatic cancer: related syndromes and clinical perspectiveSergio Carrera0Aintzane Sancho1Eider Azkona2Josune Azkuna3Guillermo Lopez-Vivanco4Hereditary Cancer Genetic Counseling Unit- Medical Oncology Department, Cruces University HospitalMedical Oncology Department, Cruces University HospitalMedical Oncology Department, Cruces University HospitalMedical Oncology Department, Cruces University HospitalMedical Oncology Department, Cruces University HospitalAbstract 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.http://link.springer.com/article/10.1186/s13053-017-0069-6Pancreatic ductal adenocarcinomaPancreatic neuroendocrine tumorFamilial pancreatic cancerGenetic testingHereditary cancer |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Sergio Carrera Aintzane Sancho Eider Azkona Josune Azkuna Guillermo Lopez-Vivanco |
spellingShingle |
Sergio Carrera Aintzane Sancho Eider Azkona Josune Azkuna Guillermo Lopez-Vivanco Hereditary pancreatic cancer: related syndromes and clinical perspective Hereditary Cancer in Clinical Practice Pancreatic ductal adenocarcinoma Pancreatic neuroendocrine tumor Familial pancreatic cancer Genetic testing Hereditary cancer |
author_facet |
Sergio Carrera Aintzane Sancho Eider Azkona Josune Azkuna Guillermo Lopez-Vivanco |
author_sort |
Sergio Carrera |
title |
Hereditary pancreatic cancer: related syndromes and clinical perspective |
title_short |
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_sort |
hereditary pancreatic cancer: related syndromes and clinical perspective |
publisher |
BMC |
series |
Hereditary Cancer in Clinical Practice |
issn |
1897-4287 |
publishDate |
2017-06-01 |
description |
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. |
topic |
Pancreatic ductal adenocarcinoma Pancreatic neuroendocrine tumor Familial pancreatic cancer Genetic testing Hereditary cancer |
url |
http://link.springer.com/article/10.1186/s13053-017-0069-6 |
work_keys_str_mv |
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