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Hereditary pancreatic neuroendocrine tumors

Fewer than five in one hundred (5%) of all pancreatic cancers start in the hormone producing cells of the pancreas. These are called neuroendocrine tumours. Of these, an even smaller number are related to 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 syndromes.

Multiple endocrine neoplasia type 1 (MEN1)

MEN1 is a family tumour syndrome caused by faults in the MEN1 gene. Multiple endocrine tumours can develop in different hormone-producing glands, including the pancreas, the parathyroid gland, and the pituitary gland. Not all tumours linked to MEN1 are cancerous. About three quarters (75%) of people with MEN1 will develop tumours in the pancreas.

People with faults in the MEN1 gene should be screened from childhood with blood tests every year, and endoscopic ultrasound (EUS) or MRI (magnetic resonance imaging) scans every three years.

The Association for Multiple Endocrine Neoplasia Disorders (AMEND) offers information and support for people with MEN1.

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 and also tumours of the kidney and the adrenal gland. Not all tumours linked with VHL are cancerous. Around half of people with VHL may have cysts in the pancreas, which don’t usually cause any symptoms. About ten to fifteen out of a hundred (10-15%) people 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 a scan of the tummy (abdominal ultrasound or MRI) every year to look for tumours in the kidneys, adrenal glands, pancreas and pelvis, starting in mid teens.

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

Neurofibromatosis type 1

This condition is caused by faults in the NF1 gene. As well as other problems, it leads to an increased risk of many tumours, including somatostatinomas of the pancreas. However, this is not common. The Neuro Foundation offers information and support for people with the condition.

Tuberous sclerosis

This is a genetic condition linked to faults in the TSC1 and TSC2 genes. It causes extra tissue growth in different organs, including the pancreas, which may occasionally become cancerous. The Tuberous Sclerosis Association offers information and support for people with the condition.

Information Standard

Published October 2015

Review date October 2017