Pancreatic Neuroendocrine Tumors (Islet Cell
Tumors)
General Information(Symptoms and Diagnosis)
Stages
Treatment
General Information About Pancreatic Neuroendocrine Tumors
(Islet Cell Tumors)
Key Points
- Pancreatic neuroendocrine tumors form in hormone-making
cells (islet cells) of the pancreas.
- Pancreatic NETs may or may not cause signs or symptoms.
- There are different kinds of functional pancreatic NETs.
- Having certain syndromes can increase the risk of
pancreatic NETs.
- Different types of pancreatic NETs have different signs and
symptoms.
- Lab tests and imaging tests are used to detect (find) and
diagnose pancreatic NETs.
- Other kinds of lab tests are used to check for the specific
type of pancreatic NETs.
- Certain factors affect prognosis (chance of recovery) and
treatment options.
Pancreatic
neuroendocrine tumors form in hormone-making cells (islet cells) of the
pancreas.
The pancreas is a gland about 6 inches long
that is shaped like a thin pear lying on its side. The wider end of the
pancreas is called the head, the middle section is called the body, and
the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine.
Anatomy of the pancreas.
The pancreas has three areas: head, body, and tail. It is found in the
abdomen near the stomach, intestines, and other organs.
There are two kinds of cells in the pancreas:
- Endocrine
pancreas cells make several kinds of hormones (chemicals that
control the actions of certain cells or organs
in the body), such as insulin
to control blood
sugar. They cluster together in many small groups (islets) throughout
the pancreas. Endocrine pancreas cells are also called islet cells or islets of
Langerhans. Tumors
that form in islet cells are called islet
cell tumors, pancreatic endocrine tumors, or pancreatic
neuroendocrine tumors (pancreatic NETs).
- Exocrine
pancreas cells make enzymes that are released
into the small intestine
to help the body digest
food. Most of the pancreas is made of ducts
with small sacs at the end of the ducts, which are lined with exocrine cells.
This summary discusses
islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic
Cancer Treatment for information on exocrine
pancreatic cancer.
Pancreatic
neuroendocrine tumors (NETs) may be benign
(not cancer) or malignant (cancer). When
pancreatic NETs are malignant, they are called pancreatic endocrine cancer
or islet cell carcinoma.
Pancreatic NETs are much
less common than pancreatic exocrine tumors and have a better prognosis.
Pancreatic
NETs may or may not cause signs or symptoms.
Pancreatic NETs may be functional or nonfunctional:
- Functional tumors make extra amounts of hormones, such as gastrin, insulin, and glucagon, that cause signs and symptoms.
- Nonfunctional tumors do not make extra amounts of hormones.
Signs and symptoms are caused by the tumor as it spreads and grows.
Most nonfunctional tumors are malignant
(cancer).
Most pancreatic NETs are
functional tumors.
There
are different kinds of functional pancreatic NETs.
Pancreatic NETs make
different kinds of hormones such as gastrin, insulin, and glucagon.
Functional pancreatic NETs include the following:
- Gastrinoma:
A tumor that forms in cells that make gastrin. Gastrin is a hormone
that causes the stomach to release an acid
that helps digest food. Both gastrin and stomach acid are increased by
gastrinomas. When increased stomach
acid, stomach ulcers,
and diarrhea are
caused by a tumor that makes gastrin, it is called Zollinger-Ellison syndrome.
A gastrinoma usually forms in the head of the pancreas and sometimes
forms in the small intestine. Most gastrinomas are malignant (cancer).
- Insulinoma:
A tumor that forms in cells that make insulin. Insulin is a hormone
that controls the amount of glucose
(sugar) in the blood. It moves glucose into the cells, where it can be
used by the body for energy. Insulinomas are usually slow-growing
tumors that rarely spread. An insulinoma forms in the head, body, or
tail of the pancreas. Insulinomas are usually benign (not cancer).
- Glucagonoma:
A tumor that forms in cells that make glucagon. Glucagon is a hormone
that increases the amount of glucose in the blood. It causes the liver to break down
glycogen. Too much glucagon causes hyperglycemia
(high blood sugar).
A glucagonoma usually forms in the tail of the pancreas. Most
glucagonomas are malignant (cancer).
- Other types of tumors: There
are other rare types of functional pancreatic NETs that make hormones,
including hormones that control the balance of sugar, salt, and water
in the body. These tumors include:
- VIPomas, which make vasoactive
intestinal peptide. VIPoma may also be called
Verner-Morrison syndrome.
- Somatostatinomas, which make somatostatin.
These other types of tumors are grouped together because they are
treated in much the same way.
Having
certain syndromes can increase the risk of pancreatic NETs.
Anything that increases
your risk of getting a disease is called a risk
factor. Having a risk factor does not mean that you will
get cancer; not having risk factors doesn't mean that you will not get
cancer. Talk with your doctor if you think you may be at risk.
Multiple endocrine neoplasia type 1
(MEN1) syndrome is a risk factor for pancreatic NETs.
Different
types of pancreatic NETs have different signs and symptoms.
Signs or symptoms can be
caused by the growth of the tumor and/or by hormones the tumor makes or
by other conditions.
Some tumors may not cause signs or symptoms. Check with your doctor if
you have any of these problems.
Signs
and symptoms of a non-functional pancreatic NET
A non-functional
pancreatic NET may grow for a long time without causing signs or
symptoms. It may grow large or spread to other parts of the body before
it causes signs or symptoms, such as:
- Diarrhea.
- Indigestion.
- A lump in the abdomen.
- Pain in the abdomen or back.
- Yellowing of the skin and whites of the eyes.
Signs
and symptoms of a functional pancreatic NET
The signs and symptoms
of a functional pancreatic NET depend on the type of hormone being
made.
Too much
gastrin may cause:
- Stomach ulcers that keep coming back.
- Pain in the abdomen, which may spread to the back. The pain
may come and go and it may go away after taking an antacid.
- The flow of stomach contents back into the esophagus (gastroesophageal reflux).
- Diarrhea.
Too much
insulin may cause:
- Low blood sugar.
This can cause blurred vision, headache, and feeling lightheaded,
tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry.
- Fast heartbeat.
Too much
glucagon may cause:
- Skin rash on the face, stomach, or legs.
- High blood sugar. This can cause headaches, frequent urination, dry skin and
mouth, or feeling hungry, thirsty, tired, or weak.
- Blood clots.
Blood clots in the lung
can cause shortness of breath, cough, or pain in the chest. Blood clots
in the arm or leg can cause pain, swelling, warmth, or redness of the
arm or leg.
- Diarrhea.
- Weight loss for no known reason.
- Sore tongue or sores at the corners of the mouth.
Too much
vasoactive intestinal peptide (VIP) may cause:
- Very large amounts of watery diarrhea.
- Dehydration.
This can cause feeling thirsty, making less urine,
dry skin and mouth, headaches, dizziness, or feeling tired.
- Low potassium
level in the blood. This can cause muscle weakness, aching, or cramps,
numbness and tingling, frequent urination, fast heartbeat, and feeling
confused or thirsty.
- Cramps or pain in the abdomen.
- Weight loss for no known reason.
Too much
somatostatin may cause:
- High blood sugar. This can cause headaches, frequent
urination, dry skin and mouth, or feeling hungry, thirsty, tired, or
weak.
- Diarrhea.
- Steatorrhea (very foul-smelling stool
that floats).
- Gallstones.
- Yellowing of the skin and whites of the eyes.
- Weight loss for no known reason.
Lab
tests and imaging tests are used to detect (find) and diagnose
pancreatic NETs.
The following tests and
procedures may be used:
- Physical
exam and history
: An exam of the body to check general signs of
health, including checking for signs of disease, such as lumps or
anything else that seems unusual. A history of the patient's health
habits and past illnesses and treatments will also be taken.
- Blood
chemistry studies : A procedure in which a
blood sample is checked to measure the amounts of certain substances,
such as glucose (sugar), released into the blood by organs and tissues in the body. An
unusual (higher or lower than normal) amount of a substance can be a
sign of disease.
- Chromogranin
A test: A test in which a blood sample is
checked to measure the amount of chromogranin A in the blood. A higher
than normal amount of chromogranin A and normal amounts of hormones
such as gastrin, insulin, and glucagon can be a sign of a
non-functional pancreatic NET.
- Abdominal
CT scan (CAT
scan): A procedure that makes a series of detailed
pictures of the abdomen, taken from different angles. The pictures are
made by a computer linked to an x-ray
machine. A dye may
be injected into a vein or swallowed to help
the organs or tissues show up more clearly. This procedure is also
called computed tomography, computerized tomography, or computerized
axial tomography.
- MRI
(magnetic resonance imaging): A procedure that uses a
magnet, radio waves,
and a computer to make a series of detailed pictures of areas inside
the body. This procedure is also called nuclear magnetic resonance
imaging (NMRI).
- Somatostatin
receptor scintigraphy : A type of radionuclide scan that may
be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that
attaches to tumors) is injected into a vein
and travels through the blood. The radioactive octreotide attaches to
the tumor and a special camera that detects radioactivity is used to
show where the tumors are in the body. This procedure is also called
octreotide scan and SRS.
- Endoscopic
ultrasound (EUS): A procedure in which an endoscope is inserted into
the body, usually through the mouth or rectum.
An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at
the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal
tissues or organs and make echoes. The echoes form a picture of body
tissues called a sonogram.
This procedure is also called endosonography.
- Endoscopic
retrograde cholangiopancreatography (ERCP):
A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the
gallbladder to the small intestine. Sometimes pancreatic cancer causes
these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope is
passed through the mouth, esophagus, and stomach into the first part of
the small intestine. An endoscope is a thin, tube-like instrument with
a light and a lens for viewing. A catheter
(a smaller tube) is then inserted through the endoscope into the
pancreatic ducts. A dye is injected through the catheter into the ducts
and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube
may be inserted into the duct to unblock it. This tube (or stent) may be left in place
to keep the duct open. Tissue samples may also be taken and checked
under a microscope
for signs of cancer.
- Angiogram
: A procedure to look at blood
vessels and the flow of blood. A contrast dye is injected
into the blood vessel. As the contrast dye moves through the blood
vessel, x-rays are taken to see if there are any blockages.
- Laparotomy
: A surgical
procedure in which an incision
(cut) is made in the wall of the abdomen to check the inside of the
abdomen for signs of disease. The size of the incision depends on the
reason the laparotomy is being done. Sometimes organs are removed or
tissue samples are taken and checked under a microscope for signs of
disease.
- Intraoperative
ultrasound : A procedure that uses
high-energy sound waves (ultrasound) to create images of internal
organs or tissues during surgery. A transducer placed directly on the
organ or tissue is used to make the sound waves, which create echoes.
The transducer receives the echoes and sends them to a computer, which
uses the echoes to make pictures called sonograms.
- Biopsy
: The removal of cells or tissues so they can be
viewed under a microscope by a pathologist
to check for signs of cancer. There are several ways to do a biopsy for
pancreatic NETs. Cells may be removed using a fine or wide needle
inserted into the pancreas during an x-ray or ultrasound. Tissue may
also be removed during a laparoscopy
(a surgical incision made in the wall of the abdomen).
- Bone
scan : A procedure to check if there are
rapidly dividing cells, such as cancer cells, in the bone. A very small
amount of radioactive material is injected into a vein and travels
through the blood. The radioactive material collects in bones where
cancer cells have spread and is detected by a scanner.
Other
kinds of lab tests are used to check for the specific type of
pancreatic NETs.
The following tests and
procedures may be used:
Gastrinoma
- Fasting serum
gastrin test: A test in which a blood sample is checked
to measure the amount of gastrin in the blood. This test is done after
the patient has had nothing to eat or drink for at least 8 hours.
Conditions other than gastrinoma can cause an increase in the amount of
gastrin in the blood.
- Basal acid output test: A
test to measure the amount of acid made by the stomach. The test is
done after the patient has had nothing to eat or drink for at least 8
hours. A tube is inserted through the nose or throat, into the stomach.
The stomach contents are removed and four samples of gastric acid are
removed through the tube. These samples are used to find out the amount
of gastric acid made during the test and the pH level of the gastric secretions.
- Secretin
stimulation test : If the basal acid
output test result is not normal, a secretin stimulation test may be
done. The tube is moved into the small intestine and samples are taken
from the small intestine after a drug
called secretin is
injected. Secretin causes the small intestine to make acid. When there
is a gastrinoma, the secretin causes an increase in how much gastric
acid is made and the level of gastrin in the blood.
- Somatostatin receptor scintigraphy:
A type of radionuclide scan that may be used to find small pancreatic
NETs. A small amount of radioactive octreotide (a hormone that attaches
to tumors) is injected into a vein and travels through the blood. The
radioactive octreotide attaches to the tumor and a special camera that
detects radioactivity is used to show where the tumors are in the body.
This procedure is also called octreotide scan and SRS.
Insulinoma
- Fasting serum glucose and insulin test:
A test in which a blood sample is checked to measure the amounts of
glucose (sugar) and insulin in the blood. The test is done after the
patient has had nothing to eat or drink for at least 24 hours.
Glucagonoma
- Fasting serum glucagon test:
A test in which a blood sample is checked to measure the amount of
glucagon in the blood. The test is done after the patient has had
nothing to eat or drink for at least 8 hours.
Other
tumor types
- VIPoma
- Serum VIP
(vasoactive intestinal peptide) test: A test in which a
blood sample is checked to measure the amount of VIP.
- Blood chemistry studies: A
procedure in which a blood sample is checked to measure the amounts of
certain substances released into the blood by organs and tissues in the
body. An unusual (higher or lower than normal) amount of a substance
can be a sign of disease. In VIPoma, there is a lower than normal
amount of potassium.
- Stool analysis
: A stool sample is checked for a higher than
normal sodium
(salt) and potassium levels.
- Somatostatinoma
- Fasting serum somatostatin test:
A test in which a blood sample is checked to measure the amount of
somatostatin in the blood. The test is done after the patient has had
nothing to eat or drink for at least 8 hours.
- Somatostatin receptor scintigraphy:
A type of radionuclide scan that may be used to find small pancreatic
NETs. A small amount of radioactive octreotide (a hormone that attaches
to tumors) is injected into a vein and travels through the blood. The
radioactive octreotide attaches to the tumor and a special camera that
detects radioactivity is used to show where the tumors are in the body.
This procedure is also called octreotide scan and SRS.
Certain
factors affect prognosis (chance of recovery) and treatment options.
Pancreatic NETs can
often be cured. The
prognosis (chance of
recovery) and
treatment options depend on the following:
- The type of cancer cell.
- Where the tumor is found in the pancreas.
- Whether the tumor has spread to more than one place in the
pancreas or to other parts of the body.
- Whether the patient has MEN1 syndrome.
- The patient's age and general health.
- Whether the cancer has just been diagnosed or has recurred (come back).
Stages of Pancreatic Neuroendocrine Tumors
Key Points
- The plan for cancer treatment depends on where the NET is
found in the pancreas and whether it has spread.
- There are three ways that cancer spreads in the body.
- Cancer may spread from where it began to other parts of the
body.
The
plan for cancer treatment depends on where the NET is found in the
pancreas and whether it has spread.
The process used to find
out if cancer has
spread within the pancreas
or to other parts of the body is called staging.
The results of the tests and procedures used to diagnose pancreatic neuroendocrine tumors
(NETs) are also used to find out whether the cancer has spread. See the
General Information section for a description of these tests and
procedures.
Although there is a
standard staging system
for pancreatic NETs, it is not used to plan treatment. Treatment of
pancreatic NETs is based on the following:
- Whether the cancer is found in one place in the pancreas.
- Whether the cancer is found in several places in the
pancreas.
- Whether the cancer has spread to lymph nodes near the
pancreas or to other parts of the body such as the liver, lung, peritoneum, or bone.
There
are three ways that cancer spreads in the body.
Cancer can spread
through tissue, the
lymph system, and
the blood:
- Tissue. The cancer spreads from where it began by growing
into nearby areas.
- Lymph system. The cancer spreads from where it began by
getting into the lymph system. The cancer travels through the lymph vessels to other parts
of the body.
- Blood. The cancer spreads from where it began by getting
into the blood. The cancer travels through the blood vessels to other parts
of the body.
Cancer
may spread from where it began to other parts of the body.
When cancer spreads
to another part of the body, it is called metastasis.
Cancer cells break
away from where they began (the primary
tumor) and travel through the lymph system or blood.
- Lymph system. The cancer gets into the lymph system,
travels through the lymph vessels, and forms a tumor (metastatic tumor) in another
part of the body.
- Blood. The cancer gets into the blood, travels through the
blood vessels, and forms a tumor (metastatic tumor) in another part of
the body.
The metastatic tumor is
the same type of tumor as the primary tumor. For example, if a
pancreatic neuroendocrine tumor spreads to the liver, the tumor cells
in the liver are actually neuroendocrine tumor cells. The disease is
metastatic pancreatic neuroendocrine tumor, not liver cancer.
Recurrent Pancreatic Neuroendocrine Tumors
Recurrent pancreatic neuroendocrine tumors
(NETs) are tumors
that have recurred (come back) after being treated. The tumors may come
back in the pancreas
or in other parts of the body.
Treatment Option Overview
Key Points
- There are different types of treatment for patients with
pancreatic NETs.
- Six types of standard treatment are used:
- Surgery
- Chemotherapy
- Hormone therapy
- Hepatic arterial occlusion or chemoembolization
- Targeted therapy
- Supportive care
- New types of treatment are being tested in clinical trials.
- Patients may want to think about taking part in a clinical
trial.
- Patients can enter clinical trials before, during, or after
starting their cancer treatment.
- Follow-up tests may be needed.
There
are different types of treatment for patients with pancreatic NETs.
Different types of
treatments are available for patients with pancreatic
neuroendocrine tumors (NETs). Some treatments are standard (the currently used
treatment), and some are being tested in clinical
trials. A treatment clinical trial is a research study meant to help
improve current treatments or obtain information on new treatments for
patients with cancer.
When clinical trials show that a new treatment is better than the
standard treatment, the new treatment may become the standard
treatment. Patients may want to think about taking part in a clinical
trial. Some clinical trials are open only to patients who have not
started treatment.
Six
types of standard treatment are used:
Surgery
An operation may be done
to remove the tumor.
One of the following types of surgery
may be used:
- Enucleation:
Surgery to remove the tumor only. This may be done when cancer occurs
in one place in the pancreas.
- Pancreatoduodenectomy:
A surgical procedure in which the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct are removed.
Enough of the pancreas is left to make digestive
juices and insulin.
The organs removed
during this procedure depend on the patient's condition. This is also
called the Whipple procedure.
- Distal
pancreatectomy: Surgery to remove the body and tail of the
pancreas. The spleen
may also be removed.
- Total gastrectomy:
Surgery to remove the whole stomach.
- Parietal cell
vagotomy: Surgery to cut the nerve
that causes stomach cells
to make acid.
- Liver resection: Surgery to remove
part or all of the liver.
- Radiofrequency
ablation: The use of a special probe with tiny electrodes
that kill cancer cells. Sometimes the probe is inserted directly
through the skin and only local
anesthesia is needed. In other cases, the probe is
inserted through an incision
in the abdomen.
This is done in the hospital with general
anesthesia.
- Cryosurgical
ablation: A
procedure in which tissue
is frozen to destroy abnormal
cells. This is usually done with a special instrument that contains
liquid nitrogen or liquid carbon
dioxide. The instrument may be used during surgery or laparoscopy or inserted
through the skin. This procedure is also called cryoablation.
Chemotherapy
Chemotherapy is a cancer
treatment that uses drugs
to stop the growth of cancer cells, either by killing the cells or by
stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs
enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When
chemotherapy is placed directly into the cerebrospinal
fluid, an organ, or a body cavity
such as the abdomen, the drugs mainly affect cancer cells in those
areas (regional chemotherapy).
Combination chemotherapy
is the use of more than one anticancer drug. The way the chemotherapy
is given depends on the type of the cancer being treated.
Hormone
therapy
Hormone therapy is a cancer
treatment that removes hormones
or blocks their action and stops cancer cells from growing. Hormones
are substances made by glands
in the body and circulated in the bloodstream. Some hormones can cause
certain cancers to grow. If tests show that the cancer cells have
places where hormones can attach (receptors),
drugs, surgery, or radiation
therapy is used to reduce the production of hormones or
block them from working.
Hepatic
arterial occlusion or chemoembolization
Hepatic arterial occlusion
uses drugs, small particles, or other agents to block or reduce the
flow of blood to
the liver through the hepatic
artery (the major blood
vessel that carries blood to the liver). This is done to
kill cancer cells growing in the liver. The tumor is prevented from
getting the oxygen
and nutrients it
needs to grow. The liver continues to receive blood from the hepatic portal vein, which
carries blood from the stomach and intestine.
Chemotherapy delivered
during hepatic arterial occlusion is called chemoembolization.
The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The
drug is mixed with the substance that blocks the artery and cuts off blood
flow to the tumor. Most of the anticancer drug is trapped near the
tumor and only a small amount of the drug reaches other parts of the
body.
The blockage may be
temporary or permanent, depending on the substance used to block the
artery.
Targeted
therapy
Targeted therapy is a type
of treatment that uses drugs or other substances to identify and attack
specific cancer cells without harming normal cells. Certain types of
targeted therapies are being studied in the treatment of pancreatic
NETs.
Supportive
care
Supportive care is given to
lessen the problems caused by the disease or its treatment. Supportive
care for pancreatic NETs may include treatment for the following:
- Stomach ulcers
may be treated with drug therapy
such as:
- Proton pump
inhibitor drugs such as omeprazole,
lansoprazole,
or pantoprazole.
- Histamine blocking drugs such as cimetidine, ranitidine, or
famotidine.
- Somatostatin-type drugs such as octreotide.
- Diarrhea
may be treated with:
- Intravenous
(IV) fluids with electrolytes such as potassium or chloride.
- Somatostatin-type drugs such as octreotide.
- Low blood sugar
may be treated by having small, frequent meals or with drug therapy to maintain a
normal blood sugar level.
- High blood sugar
may be treated with drugs taken by mouth or insulin
by injection.
New
types of treatment are being tested in clinical trials.
Information about
clinical trials is available from the NCI website.
Patients
may want to think about taking part in a clinical trial.
For some patients,
taking part in a clinical trial
may be the best treatment choice. Clinical trials are part of the
cancer research process. Clinical trials are done to find out if new
cancer treatments are safe and effective or better than the standard treatment.
Many of today's
standard treatments for cancer are based on earlier clinical trials.
Patients who take part in a clinical trial may receive the standard
treatment or be among the first to receive a new treatment.
Patients who take
part in clinical trials also help improve the way cancer will be
treated in the future. Even when clinical trials do not lead to
effective new treatments, they often answer important questions and
help move research forward.
Patients
can enter clinical trials before, during, or after starting their
cancer treatment.
Some clinical trials
only include patients who have not yet received treatment. Other trials
test treatments for patients whose cancer has not gotten better. There
are also clinical trials that test new ways to stop cancer from recurring (coming back) or
reduce the side effects
of cancer treatment.
Clinical trials are
taking place in many parts of the country. See the Treatment Options
section that follows for links to current treatment clinical trials.
These have been retrieved from NCI's
listing of clinical trials.
Follow-up
tests may be needed.
Some of the tests
that were done to diagnose
the cancer or to find out the stage
of the cancer may be repeated. Some tests will be repeated in order to
see how well the treatment is working. Decisions about whether to
continue, change, or stop treatment may be based on the results of
these tests.
Some of the tests
will continue to be done from time to time after treatment has ended.
The results of these tests can show if your condition
has changed or if the cancer has recurred
(come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Pancreatic Neuroendocrine Tumors
Gastrinoma
Treatment of gastrinoma may include
supportive care and the following:
- For symptoms
caused by too much stomach acid,
treatment may be a drug
that decreases the amount of acid made by the stomach.
- For a single tumor
in the head of the pancreas:
- Surgery
to remove the tumor.
- Surgery to cut the nerve
that causes stomach cells
to make acid and treatment with a drug that decreases stomach acid.
- Surgery to remove the whole stomach (rare).
- For a single tumor in the body or tail of the pancreas,
treatment is usually surgery to remove the body or tail of the
pancreas.
- For several tumors in the pancreas, treatment is usually
surgery to remove the body or tail of the pancreas. If tumor remains
after surgery, treatment may include either:
- Surgery to cut the nerve that causes stomach cells to
make acid and treatment with a drug that decreases stomach acid; or
- Surgery to remove the whole stomach (rare).
- For one or more tumors in the duodenum
(the part of the small intestine
that connects to the stomach), treatment is usually pancreatoduodenectomy
(surgery to remove the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct).
- If no tumor is found, treatment may include the following:
- Surgery to cut the nerve that causes stomach cells to
make acid and treatment with a drug that decreases stomach acid.
- Surgery to remove the whole stomach (rare).
- If the cancer
has spread to the liver,
treatment may include:
- Surgery to remove part or all of the liver.
- Radiofrequency
ablation or cryosurgical
ablation.
- Chemoembolization.
- If cancer has spread to other parts of the body or does not
get better with surgery or drugs to decrease stomach acid, treatment
may include:
- Chemotherapy.
- Hormone therapy.
- If the cancer mostly affects the liver and the patient has
severe symptoms from hormones
or from the size of tumor, treatment may include:
- Hepatic
arterial occlusion, with or without systemic chemotherapy.
- Chemoembolization, with or without systemic
chemotherapy.
Check the
list of NCI-supported cancer clinical trials that are now accepting
patients with gastrinoma. For more specific results, refine the search
by using other search features, such as the location of the trial, the
type of treatment, or the name of the drug. Talk with your doctor about
clinical trials that may be right for you. General information about
clinical trials is available from the NCI website.
Insulinoma
Treatment of insulinoma may include the
following:
- For one small tumor
in the head or tail of the pancreas,
treatment is usually surgery
to remove the tumor.
- For one large tumor in the head of the pancreas that cannot
be removed by surgery, treatment is usually pancreatoduodenectomy
(surgery to remove the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct).
- For one large tumor in the body or tail of the pancreas,
treatment is usually a distal
pancreatectomy (surgery to remove the body and tail of the
pancreas).
- For more than one tumor in the pancreas, treatment is
usually surgery to remove any tumors in the head of the pancreas and
the body and tail of the pancreas.
- For tumors that cannot be removed by surgery, treatment may
include the following:
- Combination
chemotherapy.
- Palliative
drug therapy to decrease the
amount of insulin
made by the pancreas.
- Hormone therapy.
- Radiofrequency ablation or cryosurgical ablation.
- For cancer that has spread to lymph
nodes or other parts of the body, treatment may include
the following:
- Surgery to remove the cancer.
- Radiofrequency
ablation or cryosurgical
ablation, if
the cancer cannot be removed by surgery.
- If the cancer
mostly affects the liver
and the patient has severe symptoms
from hormones or
from the size of tumor, treatment may include:
- Hepatic
arterial occlusion, with or without systemic chemotherapy.
- Chemoembolization,
with or without systemic chemotherapy.
Check the
list of NCI-supported cancer clinical trials that are now accepting
patients with insulinoma. For more specific results, refine the search
by using other search features, such as the location of the trial, the
type of treatment, or the name of the drug. Talk with your doctor about
clinical trials that may be right for you. General information about
clinical trials is available from the NCI website.
Glucagonoma
Treatment may include
the following:
- For one small tumor
in the head or tail of the pancreas,
treatment is usually surgery
to remove the tumor.
- For one large tumor in the head of the pancreas that cannot
be removed by surgery, treatment is usually pancreatoduodenectomy
(surgery to remove the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct).
- For more than one tumor in the pancreas, treatment is
usually surgery to remove the tumor or surgery to remove the body and
tail of the pancreas.
- For tumors that cannot be removed by surgery, treatment may
include the following:
- Combination
chemotherapy.
- Hormone therapy.
- Radiofrequency
ablation or cryosurgical
ablation.
- For cancer that has spread to lymph nodes or other parts of
the body, treatment may include the following:
- Surgery to remove the cancer.
- Radiofrequency ablation or cryosurgical ablation, if the cancer
cannot be removed by surgery.
- If the cancer
mostly affects the liver
and the patient has severe symptoms
from hormones or
from the size of tumor, treatment may include:
- Hepatic
arterial occlusion, with or without systemic chemotherapy.
- Chemoembolization,
with or without systemic chemotherapy.
Check the
list of NCI-supported cancer clinical trials that are now accepting
patients with glucagonoma. For more specific results, refine the search
by using other search features, such as the location of the trial, the
type of treatment, or the name of the drug. Talk with your doctor about
clinical trials that may be right for you. General information about
clinical trials is available from the NCI website.
Other Pancreatic Neuroendocrine Tumors
(Islet Cell Tumors)
For VIPoma, treatment
may include the following:
- Fluids
and hormone therapy
to replace fluids and electrolytes
that have been lost from the body.
- Surgery
to remove the tumor
and nearby lymph nodes.
- Surgery to remove as much of the tumor as possible when the
tumor cannot be completely removed or has spread to distant parts of
the body. This is palliative
therapy to relieve symptoms
and improve the quality of life.
- For tumors that have spread to lymph nodes or other parts
of the body, treatment may include the following:
- Surgery to remove the tumor.
- Radiofrequency
ablation or cryosurgical
ablation, if
the tumor cannot be removed by surgery.
- For tumors that continue to grow during treatment or have
spread to other parts of the body, treatment may include the following:
- Chemotherapy.
- Targeted therapy.
For somatostatinoma,
treatment may include the following:
- Surgery to remove the tumor.
- For cancer that has spread to distant parts of the body,
surgery to remove as much of the cancer as possible to relieve symptoms
and improve quality of life.
- For tumors that continue to grow during treatment or have
spread to other parts of the body, treatment may include the following:
- Chemotherapy.
- Targeted therapy.
Treatment of other types
of pancreatic neuroendocrine
tumors (NETs) may include the following:
- Surgery to remove the tumor.
- For cancer that has spread to distant parts of the body,
surgery to remove as much of the cancer as possible or hormone therapy
to relieve symptoms and improve quality of life.
- For tumors that continue to grow during treatment or have
spread to other parts of the body, treatment may include the following:
- Chemotherapy.
- Targeted therapy.
Check the
list of NCI-supported cancer clinical trials that are now accepting
patients with islet cell tumor. For more specific results, refine the
search by using other search features, such as the location of the
trial, the type of treatment, or the name of the drug. Talk with your
doctor about clinical trials that may be right for you. General
information about clinical trials is available from the NCI website.
Recurrent or Progressive Pancreatic
Neuroendocrine Tumors
(Islet Cell Tumors)
Treatment of pancreatic neuroendocrine tumors
(NETs) that continue to grow during treatment or recur (come back) may
include the following:
- Surgery
to remove the tumor.
- Chemotherapy.
- Hormone therapy.
- Targeted therapy.
- For liver metastases:
- Regional
chemotherapy.
- Hepatic
arterial occlusion or chemoembolization,
with or without systemic
chemotherapy.
- A clinical trial
of a new therapy.
-NIH
