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Islet Cell Carcinoma (Endocrine Pancreas) Treatment (PDQ®)
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Description
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What are islet cell tumors?
An islet cell tumor is a mass of abnormal cells that forms in the endocrine (hormone-producing) tissues of the pancreas. Islet cell tumors may be benign (noncancer) or malignant (cancerous). Islet cell cancer is rare.
The pancreas is about 6
inches long and is shaped like a thin pear, wider at one end and narrower at
the other. The pancreas lies behind the stomach, inside a loop formed by part
of the small intestine. The broader right end of the pancreas is called the
head, the middle section is called the body, and the narrow left end is the
tail.
The pancreas has two basic jobs in the body. It produces digestive juices that
help break down (digest) food, and hormones (such as insulin) that regulate how
the body stores and uses food. The area of the pancreas that produces
digestive juices is called the exocrine pancreas. About 95% of pancreatic
cancers begin in the exocrine pancreas. The hormone-producing area of the
pancreas has special cells called islet cells and is called the endocrine
pancreas. Only about 5% of pancreatic cancers start here. This summary has
information on cancer of the endocrine pancreas (islet cell cancer). (See
the PDQ summary on Pancreatic Cancer Treatment for more information on cancer
of the exocrine pancreas.)
The islet cells in the pancreas make many hormones, including insulin, which
help the body store and use sugars. When islet cells in the pancreas become
cancerous, they may make too many hormones. Islet cell cancers that make too
many hormones are called functioning tumors. Other islet cell cancers may not
make extra hormones and are called nonfunctioning tumors. Tumors that do not
spread to other parts of the body can also be found in the islet cells. These
are called benign tumors and are not cancer. A doctor will need to determine
whether the tumor is cancer or a benign tumor.
A doctor should be seen if there is pain in the abdomen, diarrhea, stomach
pain, a tired feeling all the time, fainting, or weight gain without eating too
much.
If there are symptoms, the doctor will order blood and urine tests to see
whether the amounts of hormones in the body are normal. Other tests, including
x-rays and special scans, may also be done.
The chance of recovery (prognosis) depends on the type of islet cell cancer the
patient has, how far the cancer has spread, and the patient’s overall health.
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Stage Explanation
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Stages of islet cell cancer
Once islet cell cancer is found, more tests will be done to find out if cancer
cells have spread to other parts of the body. This is called staging. The
staging system for islet cell cancer is still being developed. These tumors
are most often divided into one of three groups:
- islet cell cancers occurring in one site within the pancreas,
- islet cell cancers occurring in several sites within the pancreas, or
- islet cell cancers that have spread to lymph nodes near the pancreas or to
distant sites.
A doctor also needs to know the type of islet cell tumor to plan treatment.
The following types of islet cell tumors are found:
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Gastrinoma
The tumor makes large amounts of a hormone called gastrin, which causes too
much acid to be made in the stomach. Ulcers may develop as a result of too
much stomach acid.
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Insulinoma
The tumor makes too much of the hormone insulin and causes the body to store
sugar instead of burning the sugar for energy. This causes too little sugar in
the blood, a condition called hypoglycemia.
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Glucagonoma
This tumor makes too much of the hormone glucagon and causes too much sugar in the blood, a condition called hyperglycemia.
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Miscellaneous
Other types of islet cell cancer can affect the pancreas and/or small
intestine. Each type of tumor may affect different hormones in the body and
cause different symptoms.
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Recurrent
Recurrent disease means that the cancer has come back (recurred) after it has
been treated. It may come back in the pancreas or in another part of the body.
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Treatment Option Overview
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How islet cell cancer is treated
There are treatments for all patients with islet cell cancer. Three types of
treatment are used:
Surgery is the most common treatment of islet cell cancer. The doctor may take
out the cancer and most or part of the pancreas. Sometimes the stomach is
taken out (gastrectomy) because of ulcers. Lymph nodes in the area may also be
removed and looked at under a microscope to see if they contain cancer.
Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by
pill, or it may be put into the body by a needle in the vein or muscle.
Chemotherapy is called a systemic treatment because the drug enters the
bloodstream, travels through the body, and can kill cancer cells throughout the
body.
Hormone therapy uses hormones to stop the cancer cells from growing or to
relieve symptoms caused by the tumor.
Hepatic arterial occlusion or embolization uses drugs or other agents to reduce
or block the flow of blood to the liver in order to kill cancer cells growing
in the liver.
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Treatment by type
Treatment of islet cell cancer depends on the type of tumor, the stage, and the
patient’s overall health.
Standard treatment may be considered because of its effectiveness in patients
in past studies, or participation in a clinical trial may be considered. Not
all patients are cured with standard therapy and some standard treatments may
have more side effects than are desired. For these reasons, clinical trials
are designed to find better ways to treat cancer patients and are based on the
most up-to-date information. Clinical trials are ongoing in many parts of the
country for patients with islet cell cancer. To learn more about clinical
trials, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237);
TTY at 1-800-332-8615.
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Gastrinoma
Treatment may be one of the following:
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with gastrinoma.
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Insulinoma
Treatment may be one of the following:
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with insulinoma.
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Glucagonoma
Treatment may be one of the following:
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with glucagonoma.
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Miscellaneous Islet Cell Tumors
Treatment may be one of the following:
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with islet cell tumor.
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Recurrent Islet Cell Tumors
Treatment depends on many factors, including what treatment the patient had
before and where the cancer has come back. Treatment may be chemotherapy, or
patients may want to consider taking part in a clinical trial.
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent islet cell carcinoma.
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Changes to This Summary (10/04/2007)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Changes were made to this summary to match those made to the health professional version.
This information is provided from the PDQ® database in collaboration with the NCI.
