This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
A pancreatic neuroendocrine tumor, also called an islet cell tumor, is a type of neuroendocrine tumor (NET) that typically arises in the pancreas. However in some cases, a pancreatic NET occurs outside of the pancreas. A NET arises from cells that produce hormones, so the tumor can also produce hormones. It may be benign (not cancerous) or malignant (cancerous). Pancreatic NETs usually grow slowly over many years, but there are fast-growing forms.
Pancreatic NETs are called either functional or nonfunctional. A functional pancreatic NET causes specific symptoms because it makes extra hormones, such as gastrin, insulin, or glucagon. Examples of types of functional pancreatic NETs include insulinomas, glucagonomas, gastrinomas, VIPomas, and somatostatinomas. Symptoms depend on the type of hormone being made. A nonfunctional pancreatic NET generally does not cause specific symptoms, but may eventually cause symptoms relating to its location and size as it grows or spreads. Pancreatic NETs can be hard to diagnosis, often not identified until 5 to 10 years after they begin to grow.
Most pancreatic NETs are not inherited and occur sporadically in people with no family history of NETs. However, about 10% are associated with a hereditary cancer or tumor syndrome such as multiple endocrine neoplasia type 1 (MEN1), which has autosomal dominant inheritance.
Treatment options for each person depend on many factors including the type of tumor, its location, whether it has spread to other parts of the body (metastasized), and symptoms present. Treatment options may include surgery to remove all or part of the tumor (and sometimes the lymph nodes), and/or nonsurgical treatments to shrink the tumor, stop it from growing, or alleviate symptoms. The chance of recovery (prognosis) also depends upon these factors and differs from person to person.
For more information, visit GARD.