Pancreatic Cancer As A Hereditary Disease
Hereditary pancreatic cancer is not a condition the way breast cancer and other gene-related cancers are. Having a family history of the disease does increase your chances of developing pancreatic cancer slightly, but not as dramatically as does smoking. Most people have a 1 to 6 percent chance, depending on their lifestyle, of developing pancreatic cancer tumors. In people with hereditary pancreatic cancer (just another way of saying their close members have had the disease), they have about, on average, an 8 percent chance.
The National Institutes of Health breaks this number down. A person with one first-degree relative, such as a sibling or parent, who has pancreatic cancer, his or her risk of developing the disease increases by four to five times that of a person without a family history. It increases to six or seven times in people with two first-degree relatives. The number jumps to 32 times with three first-degree relatives. But given the extreme rarity of pancreatic cancer tumors, the chances of having three close relatives with the disease is tiny. Also, only about 10 percent of pancreas cancer sufferers have close blood relatives such as parents and siblings who also developed it.
Risks for developing pancreatic cancer equal to hereditary pancreatic cancer are hereditary chronic pancreatitis, hereditary nonpolyposis colorectal cancer, multiple endocrine neoplasia (many tumors of the glands), von Hippel-Lindau syndrome, familial adenomatous polyposis and Gardner syndrome, germline mutations in the BRCA2 gene, Peutz-Jeghers syndrome, and familial atypical multiple mole melanoma syndrome, a dangerous type of skin cancer.
Chronic pancreatitis, caused by the gene PRSS1, is a long-term inflammation or infection of the pancreas. Data shows that people with chronic pancreatitis had a 26-fold risk in the risk for pancreas cancer. Four percent of the study's patients developed pancreatic cancer within 20 years. Hereditary non-polyposis colorectal cancer (HNPCC), also called Lynch syndrome, makes up 5 percent of hereditary pancreatic cancer incidences. It is more of a risk factor for colorectal than pancreatic cancer. The mutated genes MLH1, MSH2, and MSH6 usually cause HNPCC. Von Hippel-Lindau syndrome is hereditary characterized by blood vessels growth. Capillaries form abnormal knots called angiomas or hemangioblastomas. They may occur in the retina, brain, spinal cord, and adrenal glands Gardner syndrome is characterized by GI polyps and multiple tumors of the bones, skin, and soft tissue. The polyps have a 100 percent chance of becoming cancerous. Hereditary breast and ovarian cancer (HBOC) syndrome is associated with the mutated genes BRCA1 and BRCA1. While BRCA1 is found in 75 percent of families with hereditary ovarian cancer, and BRCA1 increases risk for breast cancer to 85 percent, they only cause about a 10 percent chance of developing pancreatic cancer tumors. Peutz-Jeghers syndrome (PJS) is characterized by malignant intestinal polyps, spots around the lips, gums, mucous membranes, and skin. Vomiting and abdominal cramping from polyp growth also occurs. The above disorders bring as much if not more risk for developing cancer of the pancreas as does hereditary pancreatic cancer. They and hereditary pancreatic cancer are usually caused by certain mutated genes, which doctors can test for. If you think you may have a family history of the disorders, you may want to consult a genetic counselor, who will explain the pros and cons of genetic testing for the syndromes. It may improve your chances of avoiding or surviving pancreatic cancer.
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