CONTACT US
All cancer begins with a mutation in the DNA of one cell, causing the cell to grow and divide uncontrollably. Mutations that occur during a person’s lifetime, rather than inherited mutations, seem to cause most pancreatic cancers. But in some families hereditary factors play an important role. While only about 10% of pancreatic cancers are considered familial or hereditary, researchers are interested in understanding these mutations and syndromes to understand the biology of pancreatic cancer better.
Ataxia Telangiectasia (AT)
Ataxia Telangiectasia is a rare inherited condition caused by mutations in the ATM gene. Patients with AT present with a 38% lifetime risk of developing cancer. Approximately 2-3% of patients with familial pancreatic cancer have a mutation in the ATM gene.
BRCA Mutation
BRCA 1 and 2 mutations are often related to inherited breast and ovarian cancer. However, the BRCA1 mutation may also cause a small increased risk of developing pancreatic cancer. Mutations in the BRCA2 gene are associated with a 3- to 10-fold increased risk of developing pancreatic cancer. A mutation in this gene can be found in approximately 1% of individuals of Ashkenazi Jewish descent. This is higher than in other populations. People with BRCA2 mutations have a 10% lifetime risk of developing pancreatic cancer.
Cystic Fibrosis
Cystic fibrosis affects the pancreas by causing pancreatic insufficiency and chronic pancreatitis. The risk of developing pancreatic cancer is 5 to 6 times greater in people who have cystic fibrosis compared to average risk.
Familial Adenomatous Polyposis (FAP)
FAP is a rare, hereditary form of colon cancer in which a person develops hundreds to thousands of noncancerous polyps in the colon that eventually become malignant. It is associated with higher rates of thyroid, small bowel, stomach, and pancreatic cancers.
Familial Atypical Multiple Mole Melanoma (FAMMM)
FAMMM is characterized by younger age of melanoma diagnosis, many skin moles and multiple primary melanomas. People with FAMMM have a 20-to-47 fold increased risk of developing pancreatic cancer.
Hereditary Nonpolyposis Colorectal Cancer (HNPCC) or Lynch Syndrome
This is an inherited condition that is associated with 5% of colon cancer cases. Patients with HNPCC have an approximately 9-fold increased risk of developing pancreatic cancer.
Hereditary Pancreatitis
Hereditary pancreatitis is a rare, inherited condition that usually starts before age 20. It is characterized by recurrent episodes of severe inflammation of the pancreas that can lead to chronic pancreatitis and approximately a 40-55% lifetime risk of developing pancreatic cancer. Individuals with hereditary pancreatitis who also smoke may develop earlier onset pancreatic cancer.
PALB2 Mutation
About 1-3% of patients with familial pancreatic cancer have inherited mutations in the PALB2 gene. Mutations in the PALB2 gene have also been associated with an increased risk of breast cancer.
Peutz-Jeghers Syndrome
Peutz-Jeghers Syndrome is characterized by polyps in the small intestine and pigmented spots on the lips and nose. Patients with this syndrome have an 11-36% risk of developing pancreatic cancer in their lifetime.
We’re Here to Help
For free, in-depth and personalized resources and information on pancreatic cancer, contact PanCAN Patient Services.
Information provided by the Pancreatic Cancer Action Network, Inc. (“PanCAN”) is not a substitute for medical advice, diagnosis, treatment or other health care services. PanCAN may provide information to you about physicians, products, services, clinical trials or treatments related to pancreatic cancer, but PanCAN does not recommend nor endorse any particular health care resource. In addition, please note any personal information you provide to PanCAN’s staff during telephone and/or email communications may be stored and used to help PanCAN achieve its mission of assisting patients with, and finding cures and treatments for, pancreatic cancer. Stored constituent information may be used to inform PanCAN programs and activities. Information also may be provided in aggregate or limited formats to third parties to guide future pancreatic cancer research and education efforts. PanCAN will not provide personal directly identifying information (such as your name or contact information) to such third parties without your prior written consent unless required or permitted by law to do so. For more information on how we may use your information, you can find our privacy policy on our website at https://www.pancan.org/privacy/.
Loading...
