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Endocrine glands
Endocrine glands


Pancreas
Pancreas


Definition:

A pancreatic islet cell tumor is an uncommon tumor of the pancreas that arises from a type of cell called the islet cell in the pancreas.



Alternative Names:

Islet cell tumors; Islet of Langerhans tumor; Neuroendocrine tumors



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 release only one hormone that leads to specific symptoms. Pancreatic islet cell tumors can be noncancerous (benign ) or cancerous (malignant).

Islet cell tumors include:

A family history of multiple endocrine neoplasia, type I (MEN I ) is a risk factor for islet cell tumors.



Symptoms:

Symptoms are caused by the hormone the tumor is producing. For example, an insulinoma may produce too much insulin, leading to very low blood sugar levels.

Symptoms can include:



Signs and tests:

Tests may vary depending upon the symptoms. The following tests may be performed:

Occasionally, surgery is needed to diagnose and treat this condition. During this procedure, the surgeon feels the pancreas and may use ultrasound probes.



Treatment:

Treatment will depend on the type of tumor and whether the tumor is noncancerous (benign) or cancerous (malignant). Malignant tumors can spread to other organs, grow aggressively, and may not be treatable. Tumors are usually removed with surgery, if possible.

If malignant cancer 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, you may receive medications to counteract their effects. For example, with gastrinomas, the overproduction of gastrin leads to too much acid in the stomach. Medications that block acid release can reduce symptoms.



Support Groups:



Expectations (prognosis):

You may be cured if the tumors are surgically removed before they have spread to other organs. If tumors are cancerous, chemotherapy may be used, but it usually cannot cure patients.

Life-threatening problems (such as very low blood sugar) can occur due to excess hormone production, or if the cancer spreads throughout the body.



Complications:
  • Hormone crises (if the tumor releases certain types of hormones)
  • Severe low blood sugar (from insulinomas)
  • Severe ulcers in the stomach and small intestine (from gastrinomas)
  • Spread of the tumor to the liver


Calling your health care provider:

Call your health care provider if you develop symptoms of this tumor, especially if you have a family history of MEN I.



Prevention:

There is no known prevention for these tumors.




Review Date: 3/18/2008
Reviewed By: Elizabeth H. Holt, MD, PhD, Assistant Professor of Medicine, Section of Endocrinology and Metabolism, Yale University. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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