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
||The BRCA2 mutation is most often related to inherited
breast and ovarian cancer. However, approximately 7% of pancreatic
cancers are caused by inherited defects in the BRCA2 gene. 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 5% lifetime risk of developing pancreatic cancer.
||Cystic fibrosis affects the pancreas by causing
pancreatic insufficiency and chronic pancreatitis. The risk of developing
pancreatic cancer under the age of 60 is 2 times higher in people
who carry the gene mutation that causes cystic fibrosis.
|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 17% risk of developing pancreatic cancer.
||Fanconi Anemia is a rare, inherited blood disorder
that may cause a person’s bone marrow to make abnormal blood cells;
this can lead to leukemia (a type of blood cancer). People with Fanconi
Anemia have a higher risk of developing cancers of the digestive tract,
including pancreatic cancer.
|Hereditary Nonpolyposis Colorectal Cancer
(HNPCC) or Lynch Syndrome
||It is an inherited condition that is associated
with 5% of colon cancer cases. Pancreatic cancer risk may be increased
in HNPCC families in which colon cancer occurs in at least three blood
relatives, crossing at least two generations, with one of the cancer
cases occurring before the age of 50.
||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 a 30% risk of developing pancreatic cancer. Individuals
with hereditary pancreatitis who also smoke may develop earlier onset
||About 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 36% risk of developing pancreatic cancer.
|Von Hippel-Lindau Syndrome
||Von Hippel-Lindau Syndrome is a rare cancer syndrome
in which patients develop renal tumors, adrenal gland tumors and cysts.
The risk of pancreatic cancer is slightly elevated in this syndrome
and is usually associated with pancreatic neuroendocrine tumors.
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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