Educational Series/ Islet cell pancreas cancer
What is islet cell cancer?
Islet cell cancer, a rare cancer, is a disease in which cancer
(malignant) cells are found in
certain tissues of the pancreas. 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). For
more information on
cancer of the exocrine pancreas, see the PDQ patient information summary
on cancer of
the 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.
STAGE EXPLANATION
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
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:
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.
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.
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.
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.
TREATMENT OPTION OVERVIEW
How islet cell cancer is treated
There are treatments for all patients with islet cell cancer. Three
types of treatment are used:
surgery (taking out the cancer)
chemotherapy (using drugs to kill cancer cells)
hormone therapy (using hormones to stop cancer cells from growing)
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.
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.
GASTRINOMA
Treatment may be one of the following:
1. Surgery to remove the cancer.
2. Surgery to remove the stomach (gastrectomy).
3. Surgery to cut the nerve that stimulates the pancreas.
4. Chemotherapy.
5. Hormone therapy.
6. Hepatic arterial occlusion or embolization to kill cancer cells
growing in the liver.
INSULINOMA
Treatment may be one of the following:
1. Surgery to remove the cancer.
2. Chemotherapy.
3. Hormone therapy.
4. Drugs to relieve symptoms.
5. Hepatic arterial occlusion or embolization to kill cancer cells
growing in the liver.
MISCELLANEOUS ISLET CELL CANCER
Treatment may be one of the following:
1. Surgery to remove the cancer.
2. Chemotherapy.
3. Hormone therapy.
4. Hepatic arterial occlusion or embolization to kill cancer cells
growing in the liver.
RECURRENT ISLET CELL CARCINOMA
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.
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