Alternate Names: Tumor - islet cell; Islet cell tumors; Islet of Langerhan's tumor

Causes and Risks:

In the normal pancreas, cells called islet cells produce hormones that regulate a variety of bodily functions such as the blood sugar level and the production of stomach acid.

Tumors of these islet cells of the pancreas can produce any of a variety of hormones or may even produce no hormones at all. The tumors can be benign or malignant (cancerous). The tumors may produce more than one hormone, but usually one hormone predominates so that specific symptoms result.

Islet cell tumors include insulinomas, glucagonomas, gastrinomas (Zollinger-Ellison’s syndrome) and other, more rare types of tumors. Risk factors are having a family history of multiple endocrine neoplasia, Type I (MEN I).

Prevention: There is no known prevention for these tumors.

Symptoms:

• Sweating
• tremor
• rapid heart rate
• anxiety
• hunger
• dizziness
• headache
• clouding of vision
• confusion
• behavioral changes
• convulsions
• loss of consciousness
• skin rash that migrates on face, abdomen, perineum, buttocks, or lower extremities
  • may be crusty, scaly, and have raised lesions filled with clear fluid or pus
• inflamed mouth and tongue
• weight loss
• weight gain (unintentional)
• peptic ulcer pain
• vomiting blood
• diarrhea
• abdominal pain

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:

• elevated serum glucagon level
• an abdominal CT scan may reveal a pancreatic tumor (sometimes the tumor may be too small to see with a CT scan)
• elevated fasting glucose level
• abnormal glucose tolerance test
• catheterization of the pancreas to show high hormone level in the veins (this involves putting a wire into a blood vessel and taking blood out for measurements)
• MRI of abdomen to show pancreatic tumor (MRI can sometimes see smaller tumors than those seen with a CT scan)
• elevated serum insulin level
• elevated serum insulin C-peptide
• low fasting glucose level
• increased gastrin level
• positive secretin stimulation test for pancreas
• positive calcium infusion test

Treatment:

Treatment will depend upon the type of tumor discovered, and whether the tumor is benign or malignant (malignant tumors grow aggressively and may be resistant to treatment). In general, tumors are removed in an operation, if possible.

If cancerous cells spread (metastasis) to the liver, sometimes a portion of the liver will be removed as well. Some antisecretory drugs may be of benefit to reduce the production of certain hormones. H-2 (histamine-2, which produces stomach acid) blockers such as cimetidine may reduce the production of gastrin in the case of gastrinomas.

Prognosis:

If malignant and allowed to grown unchecked, islet cell tumors can metastasize to the liver or other organs. Death may result from hormone crises such as hypoglycemic reactions or from widespread metastasis.

Complications:

Metastasis (spread) of the tumor to the liver can occur. Hormone crises can occur, depending on whether or not the tumor secretes hormones and what type of hormone it makes. Gastrinomas can induce severe peptic ulcer disease.

Call your health care provider if symptoms of this tumor develop, especially if you have a family history of MEN1.