Hereditary pancreatic cancer: related syndromes and clinical perspective

Hereditary Cancer in Clinical Practice

Sergio Carrera¹  Aintzane Sancho²  Eider Azkona²  Josune Azkuna²  Guillermo Lopez-Vivanco² 

[1] Hereditary Cancer Genetic Counseling Unit- Medical Oncology Department, Cruces University Hospital;Medical Oncology Department, Cruces University Hospital;

关键词: Pancreatic ductal adenocarcinoma;    Pancreatic neuroendocrine tumor;    Familial pancreatic cancer;    Genetic testing;    Hereditary cancer;   

DOI  :  10.1186/s13053-017-0069-6

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown