What Is a Pancreatic Islet Cell Tumor?
A pancreatic islet cell tumor is a tumor that develops in the
pancreas from a type of cell called an islet cell. These cells manufacture and
release hormones, such as insulin and glucagon, into the bloodstream. An islet
cell tumor can be benign (noncancerous) or malignant (cancerous). Depending on
the type of islet cell tumor, an overproduction of certain hormones can result.
An islet cell tumor is rare, but it can cause a variety of
symptoms depending on the type of hormone that it produces. If the tumor is
malignant and spreads to other parts of the body, the cancer can become very
Other names for a pancreatic islet cell tumor are:
- islet of Langerhans tumor
- pancreatic endocrine tumor
- neuroendocrine tumor
What Are the Types of Pancreatic Islet Cell
Islet cell tumors can be either nonfunctional or functional.
Nonfunctional Islet Cell Tumors
Nonfunctional islet cell
tumors produce substances that don’t cause symptoms. The only
symptoms are those that result from the growth and spread of the tumor. These
tumors are usually malignant.
Functional Islet Cell Tumors
Functional tumors are
those that produce a hormone that results in symptoms. The three main types of
functional tumors are gastrinomas, insulinomas, and glucagonomas. Other
functional tumors exist that are extremely rare.
A gastrinoma occurs when the tumor grows from islet cells that
make the hormone gastrin. Gastrin helps release acid in the stomach for food
An insulinoma is a tumor that produces the hormone insulin.
Insulin is responsible for controlling sugar in the bloodstream.
A tumor that produces the hormone glucagon is known as a
glucagonoma. This hormone causes the liver to release glucose, or sugar, into
Rare Functional Tumors
A few types of functional islet cell tumors are extremely rare.
These include tumors that produce vasoactive intestinal peptide (VIP), which
are called VIPomas.
Somatostatinomas are another type of functional islet cell tumor
that’s extremely rare. They make the hormone somatostatin. These hormones
control sugar, water, and salt levels in the body.
What Are the Symptoms of Pancreatic Islet
The symptoms that result from having an islet cell tumor vary
depending on the type of tumor. The symptoms of nonfunctional tumors arise from
the growth and spread of the tumor. The symptoms of functional tumors depend on
the hormone produced and released into the bloodstream.
Symptoms of a Nonfunctional Tumor
The symptoms of nonfunctional tumors include:
- increased gas
- abdominal or back pain
- abdominal mass
- yellowing of the skin and the whites of the
eyes, which is called “jaundice” or “icterus”
Symptoms of a Gastrinoma
The symptoms of a gastrinoma include:
- recurring stomach ulcers
- abdominal pain that responds to antacids
- stomach contents flowing back up the esophagus
Symptoms of an Insulinoma
An insulinoma can cause an elevated heart rate and low blood
sugar. The symptoms of low blood sugar are:
- a headache
- blurred vision
Symptoms of a Glucagonoma
The symptoms of a glucagonoma include:
- a rash on the stomach, legs, or face
- unexplained weight loss
- tongue and mouth sores
A glucagonoma can cause a blood clot, which can cause the following
- shortness of breath
- chest pain
- pain in an arm or leg
- swelling of an arm or leg
- warmth of an arm or leg
A glucagonoma can also cause high blood sugar, which can cause
the following symptoms:
- dry skin
- frequent urination
Who Is at Risk for Developing a Pancreatic
Islet Cell Tumor?
An Islet cell tumor is a very rare type of tumor. The only known
risk factor for developing such a tumor is having a disease called multiple
endocrine neoplasia type 1 syndrome (MEN1) or having a family history of the
syndrome. MEN1 is an inheritable disease that causes one or more endocrine
glands to be overactive or to grow a tumor. The endocrine glands affected by
MEN1 include the pancreas, parathyroid, and pituitary gland.
How Are Pancreatic Islet Cell Tumors
There are several ways your doctor may diagnosis an islet cell
tumor. The first step is to complete an overall physical exam and review of
medical and family history. The next step is to either run a blood test or
perform an imaging technique to look for a tumor.
Blood tests look for increased levels of hormones such as
insulin, glucagon, and gastrin, as well as glucose levels in the bloodstream.
These levels can indicate an islet cell tumor.
Imaging tests allow your doctor to visualize your pancreas and
look for the presence of a tumor. These tests may include an ultrasound,
magnetic resonance imaging, or a CT scan. Another way to get an image of the
pancreas is to perform minor exploratory surgery. Your doctor may want to
insert a small camera called an endoscope to view your pancreas or to take a
small sample of the tissue for a biopsy.
What Are the Treatment Options for Pancreatic
Islet Cell Tumors?
Surgery is the most common treatment for cancerous tumors. The
purpose of the surgery is to remove as much of the tumor as possible. If the
tumor is present in other areas of your body, such as the liver, your surgeon
may remove it from those locations as well.
If the cancer is widespread throughout your body, you may need chemotherapy
to decrease the size of the tumors.
When an islet cell tumor is benign, the treatment may simply
involve medications that treat the symptoms of the overproduced hormone. A functional
tumor is typically benign. If the symptoms are severe, surgery to remove the
tumor may be necessary.
What Is the Long-Term Outlook?
If your doctor finds a cancerous tumor before it spreads beyond
the pancreas, the outlook is good. Surgery to remove it usually cures the
cancer. The more the cancer cells spread throughout the body, the lower the
chances of recovery are.
If the tumor is benign, the outlook is also good. Doctors can
usually remove the benign tumor and cure the disease through surgery.
Medications can relieve symptoms brought about by excess hormones.