Pancreatic neuroendocrine tumors (pNET) are a group of cancers that can occur in the hormone-producing cells of the pancreas. Neuroendocrine tumors of the pancreas, also called islet cell cancer, are very rare.
Neuroendocrine tumors of the pancreas start in small hormone-producing cells (islet cells) that are normally found in your pancreas.
Some pancreatic neuroendocrine tumor cells continue to secrete hormones (called functional tumors) and make too many donated hormones in your body. Examples of these types include gastrinomas and glucagonomas.
Often times, these tumors don’t secrete excessive amounts of these hormones (called non-functioning tumors).
Neuroendocrine tumors of the pancreas sometimes cause no symptoms. If so, the signs and symptoms could include:
- Stomach ulcers
- stomach pain
- Muscle spasms
- Weight loss
- Itchy skin
- Stomach or back pain
- Yellowing of the skin or eyes
It is not known what causes most neuroendocrine tumors of the pancreas.
Pancreatic neuroendocrine tumors occur when hormone-producing cells in the pancreas (islet cells) develop changes (mutations) in their DNA – the material that contains instructions for every chemical process in your body. DNA mutations cause changes in these instructions. One result is that cells grow out of control and eventually form a tumor – a mass of cancer cells. Sometimes cancer cells break off and spread to other organs, such as the liver.
Some hereditary syndromes increase your risk for this type of cancer, including:
- Multiple endocrine neoplasia, syndrome type 1 (MEN 1)
- Hippel-Lindau disease
- Von Recklinghausen disease (neurofibromatosis 1)
- Tuberous sclerosis
Factors associated with an increased risk of developing neuroendocrine tumors of the pancreas include:
- Be a man. Men are much more likely to develop these tumors than women.
- A family history of neuroendocrine tumors of the pancreas. If a family member has been diagnosed with a neuroendocrine pancreatic tumor, your risk is increased. You may have multiple endocrine neoplasms, Type 1 Syndrome (MEN 1), which increases your risk for this type of tumor.