Hereditary pancreatic neuroendocrine cancer

There are a small number of rare inherited genetic conditions that are linked to pancreatic neuroendocrine cancers.

Pancreatic neuroendocrine cancers start in neuroendocrine cells in the pancreas that produce hormones. You may also hear them called pancreatic neuroendocrine tumours or pancreatic NETs. Only about 5 in 100 (5%) pancreatic cancers are neuroendocrine cancers. Read more about pancreatic neuroendocrine cancers.

Some neuroendocrine tumours may be linked to an inherited genetic condition. These genetic conditions are called family cancer syndromes. We explain the different syndromes below. If your family is affected by one of these, ask your doctor what screening and support is available. Or contact Neuroendocrine Cancer UK, who have information and support for anyone affected by hereditary neuroendocrine cancers.

Multiple endocrine neoplasia disorders

These are inherited conditions that cause tumours in more than one endocrine gland. Endocrine glands produce hormones – the pancreas is an endocrine gland. Some types of multiple endocrine neoplasia can cause pancreatic neuroendocrine tumours.

Multiple endocrine neoplasia type 1 (MEN1) is a rare condition caused by faults in the MEN1 gene. Tumours may develop in different glands, including the pancreas, the parathyroid gland and the pituitary gland. Up to three quarters (75%) of people with MEN1 may develop pancreatic neuroendocrine cancer.

People with MEN1 should have regular monitoring (screening) to check for tumours. Check-ups should include annual blood tests and MRI, EUS and/or CT scans every 1-3 years. Read more about screening and genetic testing for pancreatic cancer.

Multiple endocrine neoplasia type 4 (MEN4) may be even rarer than MEN1, although there hasn’t been much research into it. It is caused by faults in the CDNK1B gene. It can cause tumours in the pancreas, and in the pituitary and parathyroid glands. Pancreatic neuroendocrine tumours are less common in people with MEN4 than in people with MEN1.

The Association for Multiple Endocrine Neoplasia Disorders (AMEND) offer information and support.

Von Hippel-Lindau (VHL) syndrome

Von Hippel-Lindau (VHL) syndrome is caused by a fault in the VHL gene. It can cause tumours to develop in different places in the body, including the central nervous system, kidneys, eyes and pancreas. Most tumours linked with VHL are not cancerous.

Around half of people with VHL may have cysts in the pancreas, which don’t usually cause any symptoms. Some cysts can become cancerous so cysts should be assessed to check the risk of them becoming cancer.

Up to 20 out of 100 people (20%) with VHL may develop one or more pancreatic neuroendocrine tumours. These tumours are usually non-functioning. This means they don’t produce more hormones than normal and generally don’t cause symptoms.

Screening for people with faults in the VHL gene should begin from early childhood. People diagnosed with VHL and those with a faulty VHL gene should have an ultrasound or MRI scan of their tummy every year, starting from their mid-teens. These scans check for tumours in the kidneys, adrenal glands, pancreas and pelvis. Read more about screening and genetic testing for pancreatic cancer.

VHL UK/Ireland provide information about support for people with von Hippel-Lindau syndrome.

VHL UK/Ireland provides information about support for people with von Hippel-Lindau syndrome.

Find out more about pancreatic neuroendocrine cancers from Neuroendocrine Cancer UK.

Questions about family history?

If you have questions or worries about family history of pancreatic cancer, speak to your doctor.

You can also speak to our specialist nurses on our free Support Line.

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Specialist nurse, Lisa, talks on the phone to offer support.

Published June 2022

To be reviewed June 2025