TY - JOUR
T1 - Genetic testing by cancer site
T2 - Pancreas
AU - Axilbund, Jennifer E.
AU - Wiley, Elizabeth A.
PY - 2012/7
Y1 - 2012/7
N2 - It is estimated that 5% to 10% of pancreatic cancer is familial. Although there is evidence of a major pancreatic cancer susceptibility gene, the majority of families with multiple cases of pancreatic cancer do not have an identifiable causative gene or syndrome. However, a subset of pancreatic cancer is attributable to known inherited cancer predisposition syndromes, including several hereditary breast cancer genes (BRCA1, BRCA2, and PALB2), CDKN2A, hereditary pancreatitis, hereditary nonpolyposis colorectal cancer, and Peutz-Jeghers syndrome. In addition to explaining a proportion of familial pancreatic cancer, individuals with these conditions are at increased risk for pancreatic cancer. Relatives from familial pancreatic cancer kindreds without one of these identifiable syndromes may have as high as a 32-fold risk of pancreatic cancer, depending on the number of affected first-degree relatives. Such high-risk individuals may benefit from increased surveillance, and strategies for early detection of pancreatic cancer are under evaluation.
AB - It is estimated that 5% to 10% of pancreatic cancer is familial. Although there is evidence of a major pancreatic cancer susceptibility gene, the majority of families with multiple cases of pancreatic cancer do not have an identifiable causative gene or syndrome. However, a subset of pancreatic cancer is attributable to known inherited cancer predisposition syndromes, including several hereditary breast cancer genes (BRCA1, BRCA2, and PALB2), CDKN2A, hereditary pancreatitis, hereditary nonpolyposis colorectal cancer, and Peutz-Jeghers syndrome. In addition to explaining a proportion of familial pancreatic cancer, individuals with these conditions are at increased risk for pancreatic cancer. Relatives from familial pancreatic cancer kindreds without one of these identifiable syndromes may have as high as a 32-fold risk of pancreatic cancer, depending on the number of affected first-degree relatives. Such high-risk individuals may benefit from increased surveillance, and strategies for early detection of pancreatic cancer are under evaluation.
KW - familial cancer
KW - genetics
KW - hereditary syndrome
KW - Pancreatic cancer
UR - http://www.scopus.com/inward/record.url?scp=84864622853&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84864622853&partnerID=8YFLogxK
U2 - 10.1097/PPO.0b013e3182624694
DO - 10.1097/PPO.0b013e3182624694
M3 - Article
C2 - 22846737
AN - SCOPUS:84864622853
SN - 1528-9117
VL - 18
SP - 350
EP - 354
JO - Cancer Journal (United States)
JF - Cancer Journal (United States)
IS - 4
ER -