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Pancreatic islet cell tumor
Alternative namesIslet cell tumors; Islet of Langerhans tumor; Neuroendocrine tumors
DefinitionA pancreatic islet cell tumor is an uncommon tumor of the pancreas that arises from a distinct type of cell in the pancreas, the islet cell. Normally, islet cells produce insulin and other hormones, and islet cell tumors can also produce hormones.
Causes, incidence, and risk factors
In the normal pancreas, cells called islet cells produce hormones that regulate a variety of bodily functions, such as blood sugar level and the production of stomach acid.
Tumors that arise from islet cells of the pancreas can also produce a variety of hormones, though some do not. Although islet cells produce many different hormones, most tumors secrete only one specific hormone that leads to specific symptoms. Pancreatic islet cell tumors can be benign or malignant (cancerous).
Islet cell tumors include insulinomas, glucagonomas, and gastrinomas ( Zollinger-Ellison syndrome ). A family history of multiple endocrine neoplasia, type I ( MEN I ) is a risk factor for the development of islet cell tumors.
Note: The symptoms depend upon the type of tumor and the hormone produced.
Signs and tests
The type of tests performed may vary depending upon the symptoms associated with the condition. Some of the following abnormalities may be detected on testing:
Treatment will depend upon the type of tumor discovered and whether the tumor is benign or malignant. Malignant tumors spread to other organs, grow aggressively, and may not be treatable. In general, tumors are removed surgically, if possible.
If malignant cancerous cells spread (metastasize) to the liver, a portion of the liver may also be removed, if possible. If the cancer is widespread, various forms of chemotherapy may be used to shrink the tumors.
If the abnormal production of hormones is causing problems, medications may be given to counteract their effects. For example, the overproduction of gastrin in the case of gastrinomas results in oversecretion of acid in the stomach, and medications that block acid secretion can be taken to reduce symptoms.
Patients may be cured if tumors are surgically removed before they have spread to other organs. If tumors are malignant, chemotherapy may be used, but is usually unsuccessful at curing patients. Death may result from serious problems due to excess hormone production (hormone crises), such as very low blood sugar or from widespread metastasis .
Metastasis (spread) of the tumor to the liver can occur. Hormone crises can occur, depending on whether the cells that make up the tumor secrete hormones and what type of hormones are made. Gastrinomas can induce severe ulcers in the stomach and small intestine.
Calling your health care provider
Call your health care provider if symptoms of this tumor develop, especially if you have a family history of MEN1.
PreventionThere is no known prevention for these tumors.
Update Date: 5/10/2002William Matsui, M.D., Department of Oncology, Johns Hopkins School of Medicine, Baltimore, MD. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT