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.