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2017 ; 15
(ä): 9
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Hereditary pancreatic cancer: related syndromes and clinical perspective
#MMPMID28670351
Carrera S
; Sancho A
; Azkona E
; Azkuna J
; Lopez-Vivanco G
Hered Cancer Clin Pract
2017[]; 15
(ä): 9
PMID28670351
show ga
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.