Managing pancreatic exocrine insufficiency if you have a neuroendocrine neoplasm

This leaflet has been designed to provide background information to individuals with neuroendocrine neoplasms (NENs) who require pancreatic enzyme replacement therapy (PERT).

What are neuroendocrine neoplasms?

The term neuroendocrine neoplasm (NEN) refers to any type of abnormal growth in the neuroendocrine system. The function of this system is to release hormones and communicate with other parts of the body. One of the main characteristics of neuroendocrine neoplasms is that they can release extra hormones, which can have different effects on the body.

What is pancreatic exocrine insufficiency?

One function of the pancreas, an organ near your stomach, is to release digestive enzymes that help to breakdown food. If we don’t produce enough digestive enzymes we can’t absorb the nutrients in our food. This results in undigested foods being lost in our faeces (poo). We call this pancreatic exocrine insufficiency (PEI).

Possible causes of PEI if you have neuroendocrine neoplasms

• Surgery – If you have surgery to remove your tumour, this may mean some or all of your pancreas is removed, including the area that normally produces your digestive enzymes.
• Obstructions – Some tumours can cause obstructions in the pancreas. These blockages can prevent digestive enzymes from reaching other parts of your body.
• Pancreatic atrophy – Tumours can cause nearby cells to shrink or die (become atrophic). If the cells that normally produce digestive enzymes are affected, this can cause PEI.
• Pancreatic calcification – Over time the cells in the pancreas can become calcified (a build up of calcium). This reduces the amount of enzymes released.
• Gastrinomas – A type of NEN that produces extra gastrin, which stimulates the release of extra stomach acid. This can result in the digestive enzymes being destroyed.
• Somatostatinomas – A type of NEN that produces extra somatostatin. Higher levels of somatostatin can reduce the amount of digestive enzymes released by the pancreas.