Hereditary pancreatic neuroendocrine tumors

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. Read about these scans.

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.