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Hereditary pancreatic cancers

A small number of genetic conditions are linked to an increased risk of pancreatic cancer. These are sometimes called family cancer syndromes. They are rare, and only between 5 and 10 out of 100 pancreatic cancers (5-10%) may be caused by one of these conditions.

Most family cancer syndromes are linked to pancreatic ductal adenocarcinoma, the most common type of pancreatic cancer. A very small number of family cancer syndromes are linked to neuroendocrine tumours (PancNETs) – fewer than 5 in 100 (5%) pancreatic cancers are PancNETs.

Family cancer syndromes

These rare genetic conditions can increase the risk of pancreatic cancer.

Peutz-Jeghers syndrome

This 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. This condition mainly increases the risk of bowel and breast cancer, but may also increase the risk of pancreatic cancer.

Faults in the BRCA2 and BRCA1 genes

Both men and women can have faults in the BRCA2 and BRCA1 genes. Faults in these genes greatly increase the risk of breast and ovarian cancer. They also increase the risk of other cancers, including prostate and skin cancer (melanoma).

A fault in the BRCA2 gene slightly increases the risk of pancreatic cancer. There’s less evidence that a fault in the BRCA1 gene increases the risk of pancreatic cancer. The NICE guidelines for pancreatic cancer recommend monitoring for pancreatic cancer if you have a fault in BRAC2 or BRCA1 and one or more first-degree relatives with pancreatic cancer.

Most breast, ovarian, prostate, skin and pancreatic cancers aren’t caused by faults in BRCA2 or BRCA1. So if someone in your family has one of these cancers, it doesn’t mean you have a higher risk of developing one of them.  

BRCA Umbrella has an online forum for people with a fault in a BRCA gene.

Faults in the PALB2 gene

PALB2 is a gene linked to the BRCA2 gene. A fault in this gene increases the risk of breast cancer and slightly increases the risk of pancreatic cancer.

Familial atypical multiple mole and melanoma syndrome (FAMMM)

This is a condition caused by a fault in genes called CDKN2A and CDK4. People with FAMMM have large numbers of unusual moles. They also have a higher risk of skin cancer (melanoma) and pancreatic cancer.

Lynch syndrome

Lynch syndrome is caused by faults in one of several genes: MLH1, MSH2, MSH6 or PMS2. Lynch syndrome increases the risk of bowel cancer. It also slightly increases the risk of some other cancers, including pancreatic cancer.

Lynch Syndrome UK supports people with the condition.

Li-Fraumeni syndrome

This is caused by a fault in a gene called TP53. It increases the risk of several cancers, including breast cancer, brain tumours and leukaemia (a type of blood cancer). It may also increase the risk of pancreatic cancer.

Find out more about genetic conditions

If you have one of these family cancer syndromes, you may be able to have monitoring for pancreatic cancer. Read more about the family history of pancreatic cancer.

Genetic Alliance UK provides information about genetics and links to organisations supporting people with specific genetic conditions.

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 risk of familial pancreatic cancer increases if:

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

We still don’t 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.

If you think pancreatic cancer runs in your family, speak to your doctor. If you do have a high risk of familial pancreatic cancer, you may be able to have monitoring.  

Hereditary pancreatitis

Pancreatitis is inflammation of the pancreas. Hereditary pancreatitis is a rare condition that runs in families. It starts in early childhood, and can keep coming back. It 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 severe abdominal (tummy) pain, which often needs treating in hospital. Over time, it may damage the pancreas, which can cause problems, for example with 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.

It isn’t common for pancreatitis to run in families. Chronic (long-term) pancreatitis that is caused by other things that aren’t inherited (including heavy drinking of alcohol over a long period of time) may also increase the risk of pancreatic cancer.

If you have hereditary pancreatitis, speak to your doctor. You may be able to have monitoring for pancreatic cancer.

What should I do if I think I have a family history of pancreatic cancer?

Information Standard

Updated August 2018

Review date August 2021