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Hereditary pancreatic neuroendocrine tumors

Neuroendocrine tumours (PancNETs) start in the cells of the pancreas that produce hormones. Fewer than 5 in 100 (5%) of all pancreatic cancers are PancNETs. Neuroendocrine tumours behave differently to pancreatic ductal adenocarcinoma and are treated differently.

A very small number of rare inherited genetic conditions are linked to neuroendocrine tumours. These are called family cancer syndromes.

If your family is affected by one of these family cancer syndromes, ask your doctor what screening and support is available. You can also contact the NET Patient Foundation, which has information and support for anyone affected by hereditary neuroendocrine tumours.

Multiple endocrine neoplasia

This is a rare inherited condition that causes tumours to develop in endocrine glands. Endocrine glands produce hormones.

Type 1 (MEN1) is caused by faults in the MEN1 gene. Many tumours can develop in different glands, including the pancreas, the parathyroid gland and the pituitary gland. Up to 8 out of 10 people with MEN1 (80%) are at risk of developing PancNETs.

Type 4 (MEN4) is even rarer and can also cause tumours in the pancreas. Its symptoms are similar to MEN1 but it is caused by faults in the CDNK1B gene.

Genetic screening should be offered to people with MEN1 and their first-degree relatives. Follow-up should include annual blood tests and MRI, EUS and/or CT scans every 1-3 years.

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

Von Hippel-Lindau (VHL)syndrome

This family cancer syndrome is linked to the VHL gene. It causes an increase in small blood vessels in the brain, spinal cord and back of the eyes. It also causes tumours in the kidneys and the adrenal gland. 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. About 10 to 15 out of 100  people (10-15%) with VHL also have one or more pancreatic neuroendocrine tumours. These are usually non-functioning, which means they don’t produce hormones 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 carrying a faulty VHL gene should have an ultrasound or MRI scan of the tummy every year, starting from when they are in their mid-teens. These scans check for tumours in the kidneys, adrenal glands, pancreas and pelvis.

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

Information Standard

Updated August 2018

Review date August 2021