A pancreatic islet cell tumor is a rare tumor of the pancreas that starts from a type of cell called the islet cell.
Islet cell tumors; Islet of Langerhans tumor; Neuroendocrine tumors
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:
Symptoms depend on which hormone is made by the tumor.
For example, insulinomas produce insulin, which helps the body lower blood sugar levels. Symptoms may include:
If your blood sugar gets too low, you may faint, have a seizure, or even go into a coma.
Gastrinomas make the hormone gastrin, which tells the body to make stomach acid. Symptoms may include:
Glucagonomas make the hormone glucagon, which helps the body raise blood sugar levels. Symptoms can include:
Blood tests may vary depending upon the symptoms, but may include:
The following imaging tests may be performed:
A blood sample may also be taken from a vein in the pancreas for testing.
Sometimes, surgery is needed to diagnose and treat this condition. During this procedure, the surgeon examines the pancreas by hand and with ultrasound.
Treatment depends on the type of tumor and if it is cancerous.
Cancerous tumors can grow quickly, and spread to other organs. They may not be treatable. Tumors are usually removed with surgery, if possible.
If cancer cells spread to the liver, a part of the liver may also be removed, if possible. If the cancer is widespread, chemotherapy may be used to try and shrink the tumors.
If the abnormal production of hormones is causing symptoms, 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.
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.
Call your health care provider if you develop symptoms of these tumors, especially if you have a family history of MEN I.
There is no known prevention for these tumors.
National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Neuroendocrine Tumors. National Comprehensive Cancer Network; 2011. Version 1.2011.
Jensen RT. Pancreatic endocrine tumors. In: Goldman L, Ausiello D, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011; chap 201.