The pancreas is a pear-shaped gland located in the abdomen, between the stomach and the spine. It is about six inches in length and is made up of two major components: exocrine cells and endocrine cells. The exocrine component, made up of ducts and acini (small sacs on the end of the ducts), produces enzymes, which are specialized proteins released into the small intestine that help the body digest and break down food. It is the cells lining these pancreatic ducts that most frequently become cancerous. This is called ductal adenocarcinoma of the pancreas and represents the most common subtype of pancreatic cancer; for more information, read the Cancer.Net Guide to Pancreatic Cancer.
The endocrine component of the pancreas is made up of specialized cells clustered together in islands within the organ, called islets of Langerhans. These cells produce hormones, the most critical one being insulin, an important substance that helps regulate the amount of sugar in the blood. Rarely, a tumor will form in these islet cells. An islet cell tumor can also be called a pancreatic islet cell tumor, pancreatic endocrine tumor, Islet of Langerhans tumor, or neuroendocrine tumor. An islet cell tumor can be benign (noncancerous) or malignant (cancerous), meaning it has the ability to spread to other parts of the body.
An islet cell tumor may be either functioning or nonfunctioning. There are five major classifications of functioning islet cell tumors:
Gastrinoma. A gastrinoma describes an islet cell tumor that produces a large amount of gastrin, a hormone that causes an excess amount of acid to be made in the stomach. This results in a condition called Zollinger-Ellison syndrome.
Insulinoma. This type of tumor produces too much insulin, resulting in hypoglycemia (low blood sugar). An insulinoma is more likely to be benign; only 10% become malignant.
Glucagonoma. A glucagonoma is an islet cell tumor that produces too much of the hormone glucagon. In contrast to an insulinoma, a glucagonoma causes hyperglycemia, a condition where there is too much sugar in the blood.
VIPoma. A VIPoma arises from cells in the pancreas that produce vasoactive intestinal peptide (VIP), a hormone that plays a role in water transport in the intestines. Excessive amounts of VIP can cause chronic, watery diarrhea, which causes a condition called Verner-Morrison syndrome.
Somatostatinoma. A somatostatinoma is a tumor that usually develops in the head of the pancreas. A somatostatinoma may produce somatostatin, a hormone that inhibits the secretion of several other hormones (such as growth hormone, insulin, and gastrin).
There is one major classification of nonfunctioning islet cell tumors:
Nonfunctioning tumors. Nonfunctioning tumors make up the majority of islet cell tumors. They produce none of the clinical syndromes seen above and, as a result, they are typically diagnosed at more advanced stages of disease.
Statistics
Islet cell tumors are highly treatable and uncommon, with 200 to 1,000 new cases diagnosed each year in the United States.
Cancer survival statistics should be interpreted with caution. Estimates are based on data from multiple cases of this type of cancer in the United States each year, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with an islet cell tumor. Because the survival statistics are measured in five-year (or sometimes one-year) intervals, they may not represent advances made in the treatment or diagnosis of this cancer.
A risk factor is anything that increases a person’s chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and communicating them to your doctor may help you make more informed lifestyle and health-care choices.
At this time, no factors have been identified that raise a person’s risk of developing an islet cell tumor. However, an islet cell tumor is sometimes associated with a hereditary condition called multiple endocrine neoplasia, type 1 (MEN1). MEN1 increases the risk of developing tumors of three glands: pituitary, parathyroid, and pancreas. For more information, read the Cancer.Net Guide to Multiple Endocrine Neoplasia, Type 1.
People with an islet cell tumor may experience the following symptoms. Sometimes, people with an islet cell tumor do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom on this list, please talk with your doctor.
Gastrinoma
Ulcers (as a result of increased acid)
Diarrhea
Insulinoma
Hypoglycemia, which causes fatigue (feeling tired all the time), nervousness and shakiness, dizziness or light-headedness, seizures, and fainting episodes
Confusion
Glucagonoma
Hyperglycemia, which causes frequent urination, increased thirst, and increased hunger
Rash that spreads on the face, abdomen, or lower extremities
VIPoma
Watery diarrhea
Low level of potassium in the blood
Low level of hydrochloric acid in the stomach
Flushing (redness in the face, neck, or chest)
Fatigue
Nausea
Somatostatinoma
Type 2 diabetes (sometimes called adult-onset diabetes)
Gallstones
Steatorrhea (a condition where the body cannot absorb fat, causing oily and loose stools with a particularly bad odor)
Diarrhea
Weight loss
Low level of hydrochloric acid in the stomach
Other symptoms
Abdominal pain
Jaundice (yellowing of the skin and whites of the eyes)
Doctors use many tests to diagnose a tumor and determine if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of tumors, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:
Age and medical condition
The type of tumor suspected
Severity of symptoms
Previous test results
In addition to a physical examination, the following tests may be used to diagnose an islet cell tumor:
Laboratory tests. The doctor may take samples of blood, urine, and stool to check for abnormal levels of hormones, glucose levels, and other substances.
Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail.
Ultrasound. An ultrasound uses sound waves to create a picture of the internal organs. Tumors generate different echoes of the sound waves than normal tissue does, so when the waves are bounced back to a computer and changed into images, the doctor can locate masses inside the body. During a standard (transabdominal) ultrasound, the probe is placed on the outside of the abdomen, limiting the resolution of the images. These images are often not decisive or clear enough to diagnose subtle abnormalities in the pancreas and other organs. In an endoscopic ultrasound, the probe is connected to the end of the endoscope (a thin, flexible, lighted tube that is used to look inside the body) and carefully slid down the esophagus into the stomach and duodenum (the top of the small intestine). The closer proximity to the pancreas produces a clearer image.
X-ray. An x-ray is a picture of the inside of the body. For instance, a chest x-ray can help doctors determine if the cancer has spread to the lungs. Sometimes, the patient will be asked to swallow barium (called a barium swallow), which coats the mouth and throat, to enhance the image on the x-ray.
Magnetic resonance imaging (MRI).An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. Neuroendocrine tumors of the pancreas are often best shown using an MRI, especially if they have spread to the liver.
Octreotidescan. An octreotide scan is a special type of nuclear medicine scan used to detect the position and possible spread of an islet cell tumor. A person is given a small amount of a radioactive agent, which shows up on pictures taken by a gamma camera. The test takes place over several days.
Staging is a way of describing a tumor, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the tumor's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). For example, people with a smaller tumor may not need surgery, while many people with a larger tumor do. An islet cell tumor smaller than 2 centimeters (cm) in size acts as a benign tumor almost 100% of the time.
There is no standard staging system for an islet cell tumor, so the doctor will most likely classify the tumor into one of the following three groups:
A tumor within the pancreas, but only occurring in one site
A tumor within the pancreas, occurring in multiple sites
A tumor that has spread to lymph nodes or other parts of the body
The treatment of an islet cell tumor depends on the size and location of the tumor, whether it is cancerous, whether the cancer has spread, and the person's overall health. In many cases, a team of doctors may work with the patient to determine the best treatment plan.
This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials as a treatment option when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, visit the clinical trials section.
Active Surveillance
Sometimes, an active surveillance (also called watchful waiting or watch and wait) approach without any active treatment may be recommended, as these tumors can often be quite indolent (non-aggressive) and not grow, spread, or cause problems for many months or even years. With this approach, the patient is monitored closely with regular CT scans and physical exams to determine if the tumor is growing. If the tumor shows signs of growing or becoming aggressive, active treatment would then begin.
Surgery
Surgery is the most common treatment for an islet cell tumor that is localized (has not spread outside of the pancreas). During surgery, the doctor may need to remove most or part of the pancreas, depending on the location and size of the tumor. For a gastrinoma, it may also be necessary to take out the stomach to remove ulcers, a procedure called a gastrectomy. The spleen may also need to be removed in a procedure called a splenectomy. Sometimes, an operation referred to as a Whipple procedure may be required. During a Whipple procedure, the surgeon removes the head of the pancreas and part of the small intestine, bile duct, and stomach, and then reconnects the digestive tract and biliary system.
Side effects of surgery include weakness, fatigue, and pain the first few days following the procedure. The doctor may prescribe medication to help manage these side effects. The patient will need to stay in the hospital for several days and will probably need to rest at home for about one month. It may be difficult to digest food due to the removal of all or part of the pancreas. A special diet and medicine may help. Also, the doctor can prescribe hormones and enzymes to replace those lost by the removal of the pancreas. Another side effect is the development of diabetes due to the loss of insulin, which is produced by the pancreas. Therefore, the doctor may need to prescribe insulin.
Hormone therapy
Hormone therapy may be given to relieve symptoms caused by the tumor. The most common hormone used is octreotide (Sandostatin), which is similar to one of the hormones normally produced by the body, somatostatin. The idea is that many islet cell tumors express (on their cell surface) receptors to somatostatin. Octreotide can be given either as daily injections under the skin or intramuscularly (into a muscle) on a monthly basis (called Sandostatin LAR Depot). Common side effects of octreotide include gallbladder abnormalities (gallstones and/or biliary sludge) and gastrointestinal problems, such as diarrhea and abdominal discomfort.
Chemotherapy
Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy may be given intravenously (through a vein) or orally (by mouth). The side effects of chemotherapy depend on the individual and the dose used, but can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.
The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications you've been prescribed, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions through Cancer.Net's Drug Information Resources, which provides links to searchable drug databases.
Immunotherapy
Immunotherapy (also called biologic therapy) is designed to boost the body's natural defenses to fight the cancer. It uses materials either made by the body or in a laboratory to bolster, target, or restore immune system function. Alpha interferon is a form of immunotherapy given as injections under the skin, and is sometimes used to help relieve symptoms caused by the tumor, but it can have significant side effects including fatigue, depression, and flu-like symptoms.
Hepatic artery occlusion or embolization
Generally used for patients with liver metastases (spread to the liver), these procedures block the tumor’s blood supply by temporarily sealing off the blood vessels leading to the tumor. They are usually performed by a skilled interventional radiologist and require an inpatient hospital stay. Side effects of the procedure include pain around the liver, fever, and temporary higher levels of liver enzymes as measured by blood tests. Whether this procedure is suitable for a patient depends on the size and number of tumor(s) and the location of the tumor(s) within the liver. The effectiveness of chemotherapy combined with hepatic artery embolization is still being studied.
Doctors and scientists are always looking for better ways to treat patients with an islet cell tumor. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. Patients who participate in clinical trials are among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.
Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that finding new drugs and other therapies is the only way to make progress in treating an islet cell tumor. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with an islet cell tumor.
To join a clinical trial, patients must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.
A tumor and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur.
Fear of treatment side effects is common after a diagnosis of a tumor, but it may be helpful to know that preventing and controlling side effects is a major focus of your health-care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and the person’s overall health.
Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health-care team if they do happen. Also, be sure to communicate with the doctor about side effects you experience during and after treatment. For more information on the most common side effects of cancer and different treatments, along with ways to prevent or control them, visit Cancer.Net’s section on Managing Side Effects, based on ASCO’s curriculum.
After treatment for an islet cell tumor ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years.
While there are no established guidelines for follow-up care after treatment of an islet cell tumor, people who have undergone surgery should be seen by their doctor three months after their operation for a physical examination, blood tests, and a CT scan. Follow-up thereafter should include a physical examination and blood tests approximately every six to 12 months, with additional radiographic imaging studies (such as x-rays) to be performed on an as-needed basis.
People recovering from an islet cell tumor are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about Healthy Living After Cancer.
Research about islet cell tumors is ongoing. The following advances may still be under investigation in clinical trials and may not be approved or available at this current time. Always discuss all diagnostic and treatment options with your doctor.
Antiangiogenesis research. Antiangiogenesis research, which explores how tumors develop their blood supplies and how agents disrupt these blood supplies, may benefit people with islet cell tumors. An example of an antiangiogenic agent is bevacizumab (Avastin), a monoclonal antibody that is approved for use in other cancers, such as colon cancer and lung cancer. Bevacizumab is currently being investigated in clinical trials in combination with chemotherapy for patients with advanced islet cell tumors (see drug combinations below).
Other oral medications that have antiangiogenic properties and are also being studied in people with advanced islet cell tumors include sorafenib (Nexavar), sunitinib (Sutent), pazopanib, and AMG706.
Drug combinations. Drugs used in chemotherapy, such as fluorouracil (Efudex, Adrucil, 5FU), leucovorin (Wellcovorin), capecitabine (Xeloda), oxaliplatin (Eloxatin), and temozolomide (Temodar) work in different ways to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. As noted above, several clinical trials are evaluating these chemotherapies in combination with bevacizumab.
New agents. Everolimus (RAD001), a new drug that works by inhibiting the cell cycle, is being investigated both by itself and together with other therapeutic agents in patients with advanced neuroendocrine tumors. Lanreotide (Somatuline), a somatostatin similar to octreotide, is also in clinical trial testing. Finally, a strategy that has been under active investigation for some time now involves attaching radioactive isotopes to somatostatin analogues (synthetic hormones similar to ones that the body produces naturally). When these radiolabeled agents are injected into the body, they find and destroy islet cell tumor cells by their radioactivity.
Regular communication with your doctor is important in making informed decisions about your health care. Consider asking the following questions of your doctor:
General questions:
What type of tumor do I have?
How often do you treat patients with this type of tumor?
Is it functioning or nonfunctioning? What does this mean?
Is it cancerous or benign?
Has the tumor spread outside the pancreas?
Can you explain my pathology report to me?
What are the treatment options?
What clinical trials are open to me?
What is the goal of each treatment option?
What treatment do you recommend? Why?
What are the possible side effects of each treatment option, both in the short term and the long term?
How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?
Do I need treatment right away?
For people who need surgery:
Can you explain what will happen during the surgery?
Will I need to stay in the hospital for this surgery? For how long?
What side effects will there be from my surgery?
Will this surgery affect my ability to digest food? How? For how long?
Will I need to take medicine, hormones or enzymes after this surgery? For how long?
How can other side effects be treated?
For people who need chemotherapy:
What types of chemotherapy will I receive?
How can I best prepare myself for this treatment?
What side effects can I expect from this treatment?
What will be done to treat the side effects?
After treatment:
What are the chances that the tumor will return?
What follow-up tests do I need, and how often do I need them?
What support services are available to me? To my family?
Hirshberg Foundation for Pancreatic Cancer Research
2990 S. Sepulveda Blvd., Ste. 300C
Los Angeles, CA 90064
Phone: 310-473-5121 www.pancreatic.org
Lustgarten Foundation for Pancreatic Cancer Research
1111 Stewart Ave.
Bethpage, NY 11714
Toll Free: 866-789-1000
Phone: 516-803-2304 www.lustgarten.org
Pancreatic Cancer Action Network (PanCAN)
2141 Rosecrans Ave., Ste. 7000
El Segundo, CA 90245
Toll Free: 877-272-6226
Phone: 310-725-0025 www.pancan.org
Rare Cancer Alliance
1649 N. Pacana Way
Green Valley, AZ 85614 www.rare-cancer.org
National Organization for Rare Disorders (NORD)
55 Kenosia Ave.
PO Box 1968
Danbury, CT 06813
Toll Free: 800-999-6673 (voicemail only)
Phone: 203-744-0100 www.rarediseases.org
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