All cancer begins with a mutation
in the DNA of one cell causing the cell to grow and divide uncontrollably.
Environmental, rather than inherited, factors seem to cause the genetic
mutations in 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, pancreatic cancer researchers are
interested in specific inherited genes. The following table provides the
names of the disorders that are being studied for connections to pancreatic
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 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 13 to 22 fold increased risk of developing pancreatic cancer.
|Hereditary Nonpolyposis Colorectal Cancer
(HNPCC) or Lynch Syndrome
||It is an inherited condition that is associated
with 5% of colon cancer cases. Patients with HNPCC have approximately a 9 fold increased risk of developing pancreatic cancer.
||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.
||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 is characterized by polyps in the small intestine and pigmented spots on the lips and nose. Patients with this syndrome have a 11-36% risk of developing pancreatic cancer.
Having one or more of the genetic
conditions listed above does not mean an individual will develop pancreatic
cancer. Some individuals who develop pancreatic cancer do not have any
of these risk factors. Individuals who think they may be at risk for pancreatic
cancer should discuss this with their doctor and/or genetic counselor.
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