Hereditary Pancreatic Cancer
Most cases of pancreatic cancer appear out of the blue when there is no family history of the disease. Approx. 5-10% of pancreatic cancer is hereditary meaning it runs in families and is often referred to as Familial Pancreatic Cancer (FPC). Currently there is no genetic test available for this disease.
Familial Pancreatic Cancer (FPC)
Pancreatic cancer by itself (not part of a known syndrome) runs in some families. People with first degree relatives (mother, father, brother, sister) diagnosed with pancreatic cancer have an increased risk of being in that type of family.
The greater the number of first degree relatives with pancreatic cancer the greater this risk is. For this reason, on average, a non-smoker with a first degree relative having pancreatic cancer has approximately the same increased risk of developing the disease as a tobacco user.1
In the majority of FPC families the genes that might cause pancreatic cancer are unknown. One of the aims of EUROPAC (European Registry of Hereditary Pancreatitis and Familial Pancreatic Cancer) is to identify gene changes that may increase risk in these families. For more information on EUROPAC which is based in Liverpool, UK, click here.
Pancreatitis itself is fairly common however, hereditary pancreatitis is quite rare. It is characterised by the development of recurrent episodes of severe chronic pancreatitis (inflammation of the pancreas) which starts at an early age. In most cases hereditary pancreatitis is caused by a change in the trypsinogen gene on chromosome 7. Patients with recurrent pancreatitis are recognised as being at increased risk of developing a pancreatic cancer.
Rare syndromes and pancreatic cancer risk
The syndromes outlined below are rare conditions. Unless your family has one of the following gene faults, having other types of cancer in your family is very unlikely to mean that you are at increased risk of developing pancreatic cancer. If one of these medical syndromes runs in your family it is likely you will know about it already:
Familial Breast Cancer Gene (BRCA2)
American scientists working at Johns Hopkins, Baltimore have identified that as many as 10% of pancreas cancers are caused by inherited defects in the BRCA2 gene. Gene testing for BRCA2 mutations is available.
Hereditary Colon Cancer
The HNPCC (Hereditary Non-polyposis Colorectal Cancer) syndrome is an inherited predisposition to develop colon cancer, endometrial (uterine) cancer, stomach cancer and ovarian cancer. Gene testing for HNPCC is available.
The FAMMM (Familial Atypical Multiple Mole Melanoma) syndrome is a rare hereditary syndrome which increases melanoma risk (an aggressive form of skin cancer) in some families. These patients also have an increased risk of developing pancreatic cancer.
This is an extremely rare syndrome in which affected family members develop polyps in their small intestines and pigmented spots on their lips. The polyps are an abnormal growth of tissue protruding from the normal surface of the intestine. These patients are at an increased risk of developing pancreatic and other cancers.
Ataxia telangiectasia :
Ataxia telangiectasia is a primary immunodeficiency disease which affects a number of different organs in the body. An immunodeficiency disease is one that causes the immune system to break down, making the body susceptible to diseases. It is a rare, recessive genetic disorder of childhood that occurs in between 1 out of 40,000 and 1 out of 100,000 persons worldwide.
Remember – Most pancreatic cancer appears out of the blue when there is no family history of the disease.
If you are concerned, please follow the link to download “I have a family history of PC – what are the risks and what should I do?” written by Dr Bill Greenhalf from the Liverpool Centre of EUROPAC.
Greer JB, Whitcomb DC, Brand RE (2007) Genetic predisposition to pancreatic cancer: a brief review. Am J. Gastroenterol 102: 2564-2569 ↩
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Information Product No. PCA0011v1 | Published: 03/01/2014 | Last Updated: 04/10/2016 | Next Review Due: 03/01/2017