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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?

What is familial pancreatic cancer?

Familial pancreatic cancer is pancreatic cancer that runs in families. There may be more than one close relative with pancreatic cancer on the same side of the family.

Relatives on the same side of your family are blood relatives who are directly related to you and each other by birth. They are either on your mother’s side of the family, or your father’s side of the family – not people on both sides. For example, your mother, your mother’s parents, and your children are on the same side of the family. They do not include people who are part of your wider family but not directly related to you, like your step-father, or sister’s partner.

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.

There is a higher risk of pancreatic cancer in families with a linked genetic condition and at least one family member has been diagnosed with pancreatic cancer.

What genetic conditions are linked to pancreatic cancer?

There are some rare genetic conditions that can increase the risk of pancreatic cancer. These are sometimes called family cancer syndromes.

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 genetic conditions linked to an increased risk of pancreatic neuroendocrine cancers. You may hear these cancers called pancreatic neuroendocrine tumours or NETs. Read more about these genetic conditions linked to pancreatic neuroendocrine cancers.

What genetic conditions 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 variant 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.

BRCA2 and BRCA1 genes gene variants

Both men and women can have BRCA2 and BRCA1 gene variants. Variants 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).

There is some evidence that the BRCA2 gene variant can also increase the risk of pancreatic cancer.

Some families with the BRCA2 gene variant have more cases of breast and ovarian cancer. In these families, the risk of pancreatic cancer is only slightly increased.

In families with the BRCA2 gene variant but few cases of breast or ovarian cancer, the risk of pancreatic cancer is higher.

There’s less evidence that a variant in the BRCA1 gene increases the risk of pancreatic cancer.

The NICE guidelines recommend monitoring for pancreatic cancer if you have a variant in BRCA2 or BRCA1 and and a first degree relative (parent, brother, sister or child) with pancreatic cancer.

Most pancreatic, breast, ovarian, prostate and skin cancers aren’t caused by variants 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.

PALB2 gene variants

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

The NICE guidelines recommend monitoring for pancreatic cancer if you have a variant in PALB2 and a first degree relative with pancreatic cancer.

Familial atypical multiple mole and melanoma syndrome (FAMMM)

This is a condition caused by a variant 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 recommend monitoring for pancreatic cancer if you have a variant in CDKN2A (p16) and a first degree relative with pancreatic cancer.

Lynch syndrome

Lynch syndrome is caused by variants 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 recommend that monitoring 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 variant 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 genetic conditions, you may be able to have monitoring for pancreatic cancer.
  • Use our Family History Checker to check your family risk.
  • Genetic Alliance UK provide information about genetics and links to organisations supporting people with specific genetic conditions.
  • You can also speak to your GP about a referral to a genetic testing centre.

Hereditary pancreatitis

Pancreatitis is inflammation of the pancreas. It can cause severe tummy pain, which often needs treating in hospital.

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

Worried about pancreatic cancer in your family?

Answer three quick questions to check your risk and find out if you and your family may benefit from regular monitoring.

Check your family risk
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Questions about family history?

If you have questions or worries about family history of pancreatic cancer, you can speak to our specialist nurses on our free Support Line.

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References

We have listed some of the references to the sources used to write this information. If you would like the full list of references, email us at publications@pancreaticcancer.org.uk

  • Carrera S, Sancho A, Azkona E, Azkuna J, Lopez-Vivanco G. Hereditary pancreatic cancer: related syndromes and clinical perspective. Hereditary Cancer in Clinical Practice 2017;15:9.
  • Hu C, Hart SN, Polley EC, Gnanaolivu R, Shimelis H, Lee KY, et al. Association Between Inherited Germline Mutations in Cancer Predisposition Genes and Risk of Pancreatic Cancer. Jama. 2018;319(23):2401-9.
  • Kirkegård J, Mortensen FV, Cronin-Fenton D. Chronic Pancreatitis and Pancreatic Cancer Risk: A Systematic Review and Meta-analysis. Am J Gastroenterol 2017;112(9):1366-1372.
  • Maisonneuve P and Lowenfels AB. Risk factors for pancreatic cancer: a summary review of meta-analytical studies. International Journal of Epidemiology 2015;44(1):186–198.
  • Mersch J, Jackson MA, Park M, Nebgen D, Peterson SK, Singletary C et al. Cancers Associated With BRCA1 and BRCA2 Mutations Other Than Breast and Ovarian. Cancer 2015 Jan 15;121(2):269–75. doi: 1002/cncr.29041
  • Matsubayashi H, Takaori K, Morizane C, Maguchi H, Mizuma M, Takahashi H, et al. Familial pancreatic cancer: Concept, management and issues. World J Gastroenterol 2017; 23(6):935–948.
  • National Institute for Health and Care Excellence. Pancreatic cancer in adults: diagnosis and management. NICE guideline. 2018
  • Slater EP, Langer P, Niemczyk E, Strauch K, Butler J, Habbe N, et al. PALB2 mutations in European familial pancreatic cancer families. Clinical Genetics 2010;78:490-4. doi: 1111/j.1399-0004.2010.01425.x
  • Valdez JM, Nichols KE, Kesserwan C. Li-Fraumeni syndrome: a paradigm for the understanding of hereditary cancer predisposition. Br J Haematol 2017;176(4):539–
  • Vasen H, Ibrahim I, Ponce CG, Slater EP, Matthäi E, Carrato A, Earl J, et al. Benefit of Surveillance for Pancreatic Cancer in High-Risk Individuals: Outcome of Long-Term Prospective Follow-Up Studies From Three European Expert Centers. Clin Oncol 2016;34:2010–2019.

More resources

Check your family risk

Read more

What are the symptoms of pancreatic cancer?

Read more

What causes pancreatic cancer?

Read more

Published August 2025

To be reviewed August 2028

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