Abstract

Pancreatic cancer is the fourth leading cause of cancer death in both men and women in the United States. However, it has the poorest prognosis of any major tumor type, with a 5-yr survival rate of approximately 5%. Cigarette smoking, increased body mass index, heavy alcohol consumption, and a diagnosis of diabetes mellitus have all been demonstrated to increase risk of pancreatic cancer. A family history of pancreatic cancer has also been associated with increased risk suggesting inherited genetic factors also play an important role, with approximately 5-10% of pancreatic cancer patients reporting family history of pancreatic cancer. While the genetic basis for the majority of the familial clustering of pancreatic cancer remains unclear, several important pancreatic cancer genes have been identified. These consist of high penetrance genes including BRCA2 or PALB2, to more common genetic variation associated with a modest increase risk of pancreatic cancer such as genetic variation at the ABO blood group locus. Recent advances in genotyping and genetic sequencing have accelerated the rate at which novel pancreatic cancer susceptibility genes have been identified with several genes identified within the past few years. This review addresses our current understanding of the familial aggregation of pancreatic cancer, established pancreatic cancer susceptablity genes and how this knowledge informs risk assessment and screening for high-risk families.

Links

Publisher Full Text

Authors

Klein AP

Institution

Department of Oncology and Pathology, Sol Goldman Pancreatic Cancer Research Center, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University School of Medicine, Baltimore, Maryland 21231, USA.

Source

Molecular carcinogenesis 51:1 2012 Jan pg 14-24

MeSH

Colorectal Neoplasms, Hereditary Nonpolyposis
Early Detection of Cancer
Female
Genes, BRCA1
Genes, BRCA2
Genes, Neoplasm
Genes, p16
Genetic Predisposition to Disease
Humans
Male
Nuclear Proteins
Pancreatic Neoplasms
Pancreatitis
Pedigree
Penetrance
Peutz-Jeghers Syndrome
Protein-Serine-Threonine Kinases
Risk Factors
Tumor Suppressor Proteins

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

22162228