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Hereditary pancreatic cancers

Most family cancer syndromes are linked to exocrine pancreatic cancer. Exocrine cancers make up 95 out of every 100 (95%) pancreatic cancers and include the most common type, pancreatic adenocarcinoma. There is separate information about inherited endocrine cancers here.

Family cancer syndromes

  • Peutz-Jeghers syndrome causes lots of non-cancerous growths (polyps) in the digestive system, and dark spots on the hands and face. Nine out of ten cases (90%) are caused by a fault in a gene called STK11. It increases the risk of pancreatic cancer and other cancers including bowel and breast cancers.
  • Hereditary breast and ovarian cancers are linked to faults in genes called BRCA1 and BRCA2. Faults in these genes significantly increase the risk of developing breast and ovarian cancer but they also increase the risk of other cancers, including prostate and skin cancer (melanoma). Both men and women can have faults in these genes. Faults in the BRCA2 gene slightly increase the risk of pancreatic cancer. Most breast, ovarian, prostate and skin cancers aren’t caused by BRCA1 and BRCA2 – so if you have a family member with one of these cancers, this doesn’t necessarily mean you have a higher risk of pancreatic cancer. BRCA Umbrella provide an online forum and information for people with a fault in a BRCA gene.
  • Familial atypical multiple mole and melanoma syndrome (FAMMM) is a condition caused by a fault in genes called CDKN2A and CDK4. People who carry the faulty genes have large numbers of unusual moles, and have a higher risk of both pancreatic and skin cancer (melanoma) cancer.
  • Familial adenomatous polyposis (FAP) is a bowel condition caused by a fault in a gene called APC. People with FAP have lots of polyps in their bowel and a high risk of developing bowel cancer. They also have a very slightly increased risk of pancreatic cancer.
  • Lynch syndrome (which used to be called hereditary non-polyposis colorectal cancer or HNPCC) is a condition that increases the risk of developing bowel cancer. It also increases the risk of some other cancers, including pancreatic cancer. It iscaused by faults in one of several genes, including MLH1, MSH2, MSH6 and PMS2.
  • Li-Fraumeni syndrome is due to a fault in a gene called p53. It increases the risk of cancer in most organs, including the pancreas.
  • Ataxia telangiectasia is a rare condition caused by a fault in a gene called ATM. It slightly increases the risk of pancreatic cancer.

Familial pancreatic cancer

Familial pancreatic cancer is pancreatic cancer that runs in families. This includes:

  • families with two or more first-degree relatives (parent, brother, sister or child) with pancreatic cancer
  • families with three or more relatives with pancreatic cancer on the same side of the family
  • families with a known family cancer syndrome and at least one family member with pancreatic cancer.

The chance of familial pancreatic cancer increases if:

  • relatives were diagnosed under 60
  • there are more than two people with pancreatic cancer in the family
  • there are cases of pancreatic cancer in more than one generation.

We don’t currently know what genetic faults cause most familial pancreatic cancers. We only know about the genetic faults that cause about one in five (20%) familial pancreatic cancers. The most common genetic faults that we do know about are faults on the BRCA2 gene.

If you think pancreatic cancer runs in your family, speak to your doctor about being referred to your local genetics service. They can assess your family history and they may offer genetic testing. You might also want to find out about the European Registry of Hereditary Pancreatitis and Familial Pancreatic Cancer (EUROPAC) study. This study is trying to find the genetic faults that cause familial pancreatic cancer and to develop a good way to screen for pancreatic cancer. There may be other research studies for people with a family history of pancreatic cancer. Speak to your doctor about this.

Hereditary pancreatitis

Pancreatitis is inflammation of the pancreas. Hereditary pancreatitis runs in families. It starts in early childhood, and can keep coming back. It is a rare condition and is linked to a fault in the PRSS1 gene. People who carry this faulty gene have a very high chance of developing pancreatitis. Pancreatitis can cause abdominal (tummy) pain. Over time, it may cause problems with the pancreas, such as problems digesting food.

People with hereditary pancreatitis may be more likely to get pancreatic cancer. About two fifths (40%) of people with hereditary pancreatitis may develop pancreatic cancer at some point in their lives. The risk may be higher for people who smoke and people who have diabetes. The EUROPAC study is also looking at hereditary pancreatitis.

It is not common for pancreatitis to run in families. But chronic (long-term) pancreatitis that isn’t inherited may also increase the risk of pancreatic cancer.

Information Standard

Published October 2015

Review date October 2017