Hereditary pancreatic cancers

This page explains pancreatic cancer that runs in families, and the small number of genetic conditions linked to a higher risk of pancreatic cancer.

What's in the 'Family history' section?

Family history of pancreatic cancer
Hereditary pancreatic cancers
What should I do if I think I have a family history of pancreatic cancer?
Hereditary pancreatic neuroendocrine cancer

Familial pancreatic cancer

Familial pancreatic cancer is pancreatic cancer that runs in families. Working out whether a family has this higher risk is not easy, but strong signs are:

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.

It is possible for families to have several cases of pancreatic cancer just by chance. But the more cases there are in a family, the more likely it is to be familial pancreatic cancer.

Pancreatic cancer can also run in families if a known family cancer syndrome has been diagnosed, and you have at least one family member with pancreatic cancer.

The chance that your family has familial pancreatic cancer is very low. Signs are:

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

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.

Family cancer syndromes

Family cancer syndromes are rare genetic conditions.

Most of the family cancer syndromes linked to pancreatic cancer increase the risk of pancreatic ductal adenocarcinoma. This is the most common type of pancreatic cancer. Read about these below.

There are also rare family cancer syndromes that are linked to an increased risk of pancreatic neuroendocrine cancers. You may hear these cancers called pancreatic neuroendocrine tumours or NETs. Read more about family cancer syndromes linked to pancreatic neuroendocrine cancers.

What family cancer syndromes are linked to pancreatic ductal adenocarcinoma?

These rare genetic conditions can increase the risk of pancreatic cancer. Find out what to do if you have one of these genetic conditions.

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.

The NICE guidelines for pancreatic cancer recommend monitoring for pancreatic cancer if you have Peutz-Jeghers syndrome.

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).

In families with one or more cases of pancreatic cancer (but few cases of breast or ovarian cancer), a fault in the BRCA2 gene increases the risk of pancreatic cancer. In families with breast and ovarian cancer, 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 BRCA2 or BRCA1 and one or more first degree relatives (parent, brother, sister or child) with pancreatic cancer.

Most pancreatic, breast, ovarian, prostate and skin 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.

The NICE guidelines for pancreatic cancer recommend monitoring for pancreatic cancer if you have a fault in PALB2 and one or more first degree relatives with pancreatic cancer.

Familial atypical multiple mole and melanoma syndrome (FAMMM)

This is a condition caused by a fault in genes called CDKN2A (p16) and CDK4. People with FAMMM have large numbers of unusual moles. They also have a higher risk of skin cancer (melanoma) and pancreatic cancer.

The NICE guidelines for pancreatic cancer recommend monitoring for pancreatic cancer if you have a fault in CDKN2A (p16) and one or more first degree relatives with 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. You may hear of Lynch I or Lynch II. Lynch II is the form of the syndrome that may be linked with pancreatic cancer.

The NICE guidelines for pancreatic cancer recommend that monitoring for pancreatic cancer should be considered if you have Lynch syndrome and any first degree relatives with pancreatic cancer.

Lynch Syndrome UK supports people with the condition.

Li-Fraumeni syndrome

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

Hereditary pancreatitis

Pancreatitis is inflammation of the pancreas. It can cause severe tummy pain, which often needs treating in hospital. Over time it may damage the pancreas, which can cause problems, for example with digesting food.

Hereditary pancreatitis is pancreatitis that runs in families. It is rare. It is linked to a fault in the PRSS1 gene. People who have this faulty gene have a very high chance of developing pancreatitis. The pancreatitis starts in early childhood and can keep coming back.

People with hereditary pancreatitis may be more likely to get pancreatic cancer. About 2 in 5 (40%) 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.

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

Hereditary pancreatitis may lead to chronic (long term) pancreatitis. But in most cases chronic pancreatitis is caused by other things that aren’t inherited. Chronic pancreatitis may also increase the risk of pancreatic cancer, although the risk is low.