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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.[14645] It may be benign (not cancerous) or malignant (cancerous). Pancreatic NETs usually grow slowly over many years, but there are fast-growing forms.[14645]
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.[14645][14646] Examples of types of functional pancreatic NETs include insulinomas, glucagonomas, gastrinomas, VIPomas, and somatostatinomas.[14645] Symptoms depend on the type of hormone being made.[14646] 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.[14646] Pancreatic NETs can be hard to diagnosis, often not identified until 5 to 10 years after they begin to grow.[14645]
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.[14647]
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.[14645] 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.[14645] The chance of recovery (prognosis) also depends upon these factors and differs from person to person.[14646]
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