Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®): Treatment - Patient Information [NCI] - General Information About Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)
Pancreatic neuroendocrine tumors form in hormone-making cells (islet cells) of the pancreas.
The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine.
Anatomy of the pancreas. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs.
There are two kinds of cells in the pancreas:
- Endocrine pancreas cells make several kinds of hormones (chemicals that control the actions of certain cells or organs in the body), such as insulin to control blood sugar. They cluster together in many small groups (islets) throughout the pancreas. Endocrine pancreas cells are also called islet cells or islets of Langerhans.
- Exocrine pancreas cells make enzymes that are released into the small intestine to help the body digest food. Most of the pancreas is made of ducts with small sacs at the end of the ducts, which are lined with exocrine cells. This summary discusses islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic Cancer Treatment for information on exocrine pancreatic cancer.
A pancreatic neuroendocrine tumor (NET) may also be called a pancreatic endocrine tumor (PET), islet cell tumor, islet cell carcinoma, or pancreatic carcinoid.
Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis.
Pancreatic NETs may or may not cause symptoms.
Pancreatic NETs may be functional (the hormones that are released cause symptoms) or nonfunctional (the hormones that are released do not cause symptoms) tumors:
- Functional tumors make one or more hormones, such as gastrin, insulin, and glucagon, that cause symptoms. Most functional tumors are benign (not cancer).
- Nonfunctional tumors make substances that do not cause symptoms. Symptoms are caused by the tumor as it spreads and grows. Most nonfunctional tumors are malignant (cancer).
Most pancreatic NETs are functional tumors.
There are different kinds of functional pancreatic NETs.
Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following:
- Gastrinoma: A tumor that forms in cells that make gastrin. Gastrin is a hormone that causes the stomach to release an acid that helps digest food. Both gastrin and stomach acid are increased by gastrinomas. When increased stomach acid, stomach ulcers, and diarrhea are caused by a tumor that makes gastrin, it is called Zollinger-Ellison syndrome. A gastrinoma usually forms in the head of the pancreas and sometimes forms in the small intestine. Most gastrinomas are malignant (cancer).
- Insulinoma: A tumor that forms in cells that make insulin. Insulin is a hormone that controls the amount of glucose (sugar) in the blood. It moves glucose into the cells, where it can be used by the body for energy. Insulinomas are usually slow-growing tumors that rarely spread. An insulinoma forms in the head, body, or tail of the pancreas. Insulinomas are usually benign (not cancer).
- Glucagonoma: A tumor that forms in cells that make glucagon. Glucagon is a hormone that increases the amount of glucose in the blood. It causes the liver to break down glycogen. Too much glucagon causes hyperglycemia (high blood sugar). A glucagonoma usually forms in the tail of the pancreas. Most glucagonomas are malignant (cancer).
- Other types of tumors: There are other rare types of functional pancreatic NETs that make hormones, including hormones that control the balance of sugar, salt, and water in the body. These tumors include:
These other types of tumors are grouped together because they are treated in much the same way.
- VIPomas, which make vasoactive intestinal peptide. VIPoma may also be called Verner-Morrison syndrome.
- Somatostatinomas, which make somatostatin.