Hereditary Pancreas Cancer
Understanding Genetic Risk for Pancreatic Cancer
Most of the time, pancreas cancer happens by chance or because of environmental influences. Sometimes, pancreas cancer can be hereditary. This means that an increased risk to develop pancreas cancer is passed down in a family. About 5-10% of pancreas cancer is caused by an inherited risk. There are many genes associated with inherited pancreas cancer risk. The genes specifically associated with pancreas adenocarcinoma include:
| Gene Name | Syndrome | Associated Cancers |
|---|---|---|
BRCA1, BRCA2 | Hereditary Breast and Ovarian Cancer syndrome | Breast, ovarian, prostate, pancreas, melanoma |
MLH1, MSH2, MSH6, PMS2, EPCAM | Lynch syndrome | Colon, uterine, stomach, ovarian, pancreas, small bowel, urinary tract |
APC | Familial Adenomatous Polyposis | Colon, thyroid, pancreas |
CDKN2A, CDK4 | Familial Atypical Multiple-Mole Melanoma syndrome | Melanoma, pancreas |
STK11 | Peutz-Jeghers syndrome | Small bowel, gastric, breast, lung, pancreas |
ATM | Not yet named | Breast, pancreas |
PALB2 | Not yet named | Breast, pancreas |
TP53 | Li Fraumeni syndrome | Soft tissue and osteosarcomas, breast cancer, brain, adrenocortical carcinoma, |
Researchers are working to better define the risk for pancreas cancer associated with each of the above syndromes and to develop screening and prevention recommendations for individuals at elevated risk. At BIDMC, the pancreas cancer specialists work with the Cancer Genetics and Prevention Program to make individualized management plans for individuals and families who have an elevated risk to develop pancreas cancer.