Pancreas Cancer

Overview

Pancreas cancer is a disease in which normal cells in the pancreas malfunction and begin to grow uncontrollably. These cancerous cells can build up and form a mass, called a tumor.  As it grows, a pancreatic tumor can affect the functioning of the pancreas, grow outward to involve nearby blood vessels and organs, and eventually metastasize (spread) to other parts of the body.

Function of the pancreas

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 composed of two major components:

• The exocrine component, made up of ducts and acini (small sacs on the end of the ducts), produces enzymes (specialized proteins) that are released into the small intestine to help the body digest and break down food, particularly fats.

• 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 specific hormones, with the most important one being insulin, a substance that helps control the amount of sugar in the blood.

Types of pancreatic cancer

The most common type of pancreatic cancer is called ductal adenocarcinoma, or simply, adenocarcinoma. In this type, the cancer begins in the exocrine component. The remainder of this section focuses on adenocarcinoma.

Much less commonly, tumors can begin in the islets of Langerhans, the endocrine component. These are known as islet cell tumors or pancreatic neuroendocrine tumors. There are several subtypes of pancreatic endocrine tumors. Some neuroendocrine tumors grow without producing chemicals (hormones) and are called non-functioning tumors. Others may produce hormones that cause metabolic imbalances, such as a very low blood sugar level (insulin-producing tumors, or insulinomas), or a very high blood sugar level (glucagonomas), or other problems like severe diarrhea (a symptom of VIPomas, which produce a substance called vasoactive intestinal peptide). It is important to distinguish a neuroendocrine tumor from an adenocarcinoma of the pancreas because each acts and is treated differently from one another. For more information, read the Guide to Islet Cell Tumors.

Find out more about basic cancer terms used in this section.

Statistics

In 2010, an estimated 43,140 adults (21,370 men and 21,770 women) in the United States will be diagnosed with pancreatic cancer. It is estimated that 36,800 deaths (18,770 men and 18,030 women) from this disease will occur this year. Pancreatic cancer is the tenth most common cancer in men and women, and the fourth leading cause of cancer death in men and women. As explained above, most pancreatic cancers are adenocarcinomas, and it is to that type of pancreatic cancer that the following statistics apply.

Because pancreatic cancer often does not cause specific symptoms early on in its development, it is often not detected until the cancer has metastasized beyond the pancreas to other areas of the body. The overall one-year relative survival rate (percentage of people who survive at least one year after the cancer is detected, excluding those who die from other diseases) of people with pancreatic cancer is 25%, and the five-year relative survival rate (percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases) is approximately 6%. If the cancer is detected at an early stage where surgical resection (surgical removal of the tumor) is possible, the five-year relative survival rate is about 22%.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of 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 pancreatic cancer. Because the survival statistics are measured in one-year or five-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer.

Learn more about understanding statistics.

Statistics adapted from the American Cancer Society's publication, Cancer Facts & Figures 2010.

Risk Factors

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.

The following factors may raise a person’s risk of developing pancreatic cancer:

Age. The risk of developing pancreatic cancer increases with age. Most pancreatic cancers occur in people older than 60.

Gender. More men are diagnosed with pancreatic cancer than women.

Race. Black people are more likely than Asians, Hispanics, or white people to develop pancreatic cancer.

Smoking. Smokers are two to three times more likely to develop pancreatic cancer than nonsmokers.

Obesity and diet. Regularly eating foods high in fat is a risk factor for pancreatic cancer. Research has shown that obese and even overweight men and women have a higher risk of dying from pancreatic cancer.

Diabetes. The sudden onset of type 2 diabetes can be an early symptom of pancreatic cancer. Whether diabetes itself is a risk factor for the development of pancreatic cancer has been a topic of great interest for many years with large studies reaching different conclusions. It is now believed that long-term diabetes does increase an individual’s risk of developing pancreatic cancer to a modest degree.

Family history. A person’s chance of developing this cancer increases three-fold if a first-degree relative (mother, father, sister, or brother) developed pancreatic cancer. That risk increases even further if more first-degree relatives are affected. Also, melanoma that runs in families and certain hereditary forms of colon, breast, and ovarian cancers are associated with an increased risk of developing pancreatic cancer. Read more about the genetics of pancreatic cancer.

Chronic pancreatitis. Pancreatitis is the inflammation of the pancreas, a painful disease of the pancreas. Some research suggests that having chronic pancreatitis may increase the risk of developing pancreatic cancer.

Hereditary pancreatitis. For more information on this condition, visit the Guide to Hereditary Pancreatitis.

Chemicals. Exposure to certain chemicals (such as pesticides, benzene, certain dyes, and petrochemicals) may increase the risk of developing pancreatic cancer.

Hepatitis B infection. Hepatitis viruses are viruses that infect the liver. One recent study has shown that evidence of a previous hepatitis B infection was twice as common in people with pancreatic cancer than in people without the cancer. More research is needed to evaluate the nature of this link and to confirm the results of this study.

Symptoms

People with pancreatic cancer may experience the following symptoms. Sometimes, people with pancreatic cancer 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.

Doctors often refer to pancreatic cancer as a silent disease because it usually does not cause any noticeable symptoms in the beginning. Also, there are currently no blood tests that can reliably detect the cancer while it is in its early stage. As the cancer grows, symptoms may include:

• Yellow skin and eyes, darkening of the urine, itching, and clay-colored stool, which are signs of obstructive jaundice (blockage of the bile ducts)

• Pain in upper abdomen or upper back

• Painful swelling of an arm or leg due to a blood clot

• Burning feeling in stomach or other gastrointestinal discomforts

• Floating stools with a particularly bad odor, due to malabsorption of fats

• Weakness

• Loss of appetite

• Nausea and vomiting

• Unexplained weight loss

Diagnosis

Doctors use many tests to diagnose cancer and determine if it has metastasized. Some tests may also determine which treatments may be the most effective. For most types of cancer, 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 cancer suspected

• Severity of symptoms

• Previous test results

If a doctor suspects that a person has pancreatic cancer, he or she will first ask about the person's medical history and examine the person to look for signs of the disease. The following tests may be used to diagnose pancreatic cancer:

Physical examination. The doctor will examine the skin and eyes to see if they are yellow, which is a sign of jaundice. Jaundice can result from a tumor at the head of the pancreas obstructing normal flow of bile (a substance produced in the liver) into the small intestine. The doctor will also feel the abdomen for changes caused by the cancer, although the pancreas itself, located in the back of the upper abdomen, is rarely palpable, meaning the doctor cannot feel it. The presence of ascites (an abnormal buildup of fluid in the abdomen) may be another sign of cancer.

Laboratory tests. The doctor may take samples of blood to check for abnormal levels of bilirubin and other substances. Bilirubin is a chemical that may reach high levels in patients with pancreatic cancer due to blockage of the common bile duct by a tumor. There are many other non-cancerous causes of an elevated bilirubin level, such as hepatitis, gall stones, or mononucleosis. CA 19-9 is a tumor marker (substances in the body that may be found at higher levels if cancer is present) that can be measured in the blood, and is frequently elevated in individuals with pancreatic cancer. An elevated CA 19-9 test by itself should not be used to make the diagnosis of pancreatic cancer, as CA 19-9 can be elevated in other, noncancerous conditions as well (such as pancreatitis, cirrhosis of the liver, and blockage of the common bile duct).

Imaging procedures allow doctors to determine where the cancer is located and whether it has spread from the pancreas to other areas of the body. However, pancreatic cancer often does not develop as a single large tumor mass, and therefore can sometimes be difficult to see on imaging tests.

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. A contrast medium (a special dye) is usually injected into a patient’s vein to provide better detail.

Ultrasound. An ultrasound uses sound waves to create a picture of the internal organs. There are two types of ultrasound devices: transabdominal and endoscopic.

• A transabdominal ultrasound device is placed on the outside of the abdomen and is slowly moved around by the doctor to produce an image of the pancreas and surrounding structures.

• The endoscopic ultrasound (EUS) device consists of a thin, lighted tube that is passed through the patient's mouth and stomach and down into the small intestine in order to take a picture of the pancreas. This procedure is very specialized and requires a gastroenterologist (a doctor who specializes in the function and disorders of the gastrointestinal tract, including stomach, intestines, and associated organs) who has special training in this area. It is generally done under sedation, so the patient sleeps through the procedure.

Endoscopic retrograde cholangiopancreatography (ERCP). In this procedure performed by a gastroenterologist, an endoscope (a thin, lighted tube) is passed into the small intestine through the mouth and stomach. A catheter (smaller tube) is passed through the endoscope and into the bile ducts and pancreatic ducts. Dye is injected into the ducts, and the doctor then takes x-rays that can show whether a duct is compressed or narrowed. Often, a plastic or metal stent can be placed across the obstructed bile duct during ERCP to help relieve any jaundice. Brushings of the tissue, which means obtaining samples of the tissue during this procedure, sometimes help confirm the diagnosis of cancer. The patient is lightly sedated during this procedure.

Percutaneous transhepatic cholangiography (PTC). In this x-ray procedure, a thin needle is inserted through the skin and into the liver. A dye is injected through the needle, so the bile ducts show up on x-rays. By looking at the x-rays, the doctor can tell whether there is a blockage of the bile ducts.

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).

One biopsy technique to obtain pancreas tissue is fine needle aspiration, in which a needle is inserted into the pancreas to aspirate (suction out) cells. An x-ray or CT-guided ultrasound is used to help guide the needle. Other ways to collect a sample of pancreas tissue involve the use of ERCP, EUS, or surgery. If the cancer has spread to other organs, a biopsy may be obtained from one of these other sites (such as the liver). A surgical biopsy can be performed either by opening the abdomen or by using a laparoscopic approach to provide openings for a camera and surgical instruments, which requires much smaller incisions.

Some patients, when having surgery or certain types of biopsies, elect to have some of their leftover tissue specimen frozen and sent to independent laboratories to have genetic and molecular profiles performed. This process may help predict which treatments may work best. The use of biospecimens in this manner has not been fully confirmed scientifically in research studies. However, with the increase in new drugs called targeted therapies (see Current Research), it is an area of increasing interest and scientific focus. It is important to note that many insurance companies do not reimburse for these types of tests yet. Talk with your doctor for more information.

Learn more about what to expect when having common tests, procedures, and scans.

Find out more about common terms used during a diagnosis of cancer.

Staging

Staging is a way of describing a cancer, 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 cancer'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). There are different stage descriptions for different types of cancer.

Doctors use several systems to stage pancreatic cancer. The method used to stage other cancers, the TNM classification, is not routinely applied to pancreatic cancer; however, for completeness, it is discussed below. A more common classification is one that places pancreatic cancer into three categories:

Resectable. This type of pancreatic cancer can be surgically removed. A tumor may lie within the pancreas or extend beyond it, but there is no involvement of the critical arteries or veins in the area. There is no evidence of any spread to areas outside of the pancreas. Approximately 10% to 15% of patients are diagnosed at this stage.

Locally advanced. This type is still confined to the area around the pancreas, but cannot be surgically removed because there is involvement of the critical arteries or veins, or the tumor directly extends to involve surrounding organs. There is no evidence of spread to any distant areas of the body. Approximately 35% to 40% of patients are diagnosed at this stage.

Metastatic. The tumor has spread beyond the area of the pancreas and involves other organs, such as the liver or distant areas of the abdomen. Approximately 45% to 55% of patients are diagnosed at this stage.

By classifying each cancer into one of these three categories, the health care team can then plan the best treatment strategy.

TNM Staging System

Doctors frequently use a tool called the TNM system to stage other types of cancer. Because doctors generally classify a tumor during surgery, and because many patients with pancreatic cancer do not undergo surgery, the TNM system is not used as much with pancreatic cancer as it is in other diseases.

The TNM system uses three criteria to judge the stage of the cancer: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to the rest of the body. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.

TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:

• How large is the primary tumor and where is it located? (Tumor, T)

• Has the tumor spread to the lymph nodes? (Node, N)

• Has the cancer metastasized to other parts of the body? (Metastasis, M)

Tumor. Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the size and location of the tumor. This helps the doctor develop the best treatment plan for each patient. Specific tumor stage information listed below.

TX: The primary tumor cannot be evaluated.

T0: No evidence of cancer was found in the pancreas.

Tis: Refers to carcinoma in situ (which is very early cancer that has not spread.)

T1: The tumor is in the pancreas only, and it is 2 centimeters (cm) or smaller in size.

T2: The tumor is in the pancreas only, and it is larger than 2 cm.

T3: The tumor extends beyond the pancreas, but the tumor does not involve the major arteries or veins near the pancreas.

T4: The tumor extends beyond the pancreas into major arteries or veins near the pancreas. A T4 tumor is unresectable (unable to be completely removed during surgery).

Node. The "N" in the TNM staging system is for lymph nodes. Lymph nodes are tiny, bean-shaped organs located throughout the body that normally help fight infection and disease as part of the body's immune system. In pancreatic cancer, regional lymph nodes are those lymph nodes near the pancreas and distant lymph nodes are those lymph nodes in other parts of the body.

NX: The regional lymph nodes cannot be evaluated.

N0: The cancer was not found in the regional lymph nodes.

N1: The cancer has spread to regional lymph nodes.

Distant metastasis. The "M" in the TNM system indicates whether the cancer has spread to other parts of the body.

MX: Distant metastasis cannot be evaluated.

M0: The disease has not metastasized.

M1: There is metastasis to another part of the body, including distant lymph nodes. Distant spread of pancreatic cancer occurs mainly in the liver, peritoneum (lining of the abdominal cavity), and lungs.

Cancer stage grouping

Doctors assign the stage of the cancer by combining the T, N, and M classifications.

Stage 0: Refers to cancer in situ, in which the cancer has not yet invaded outside the duct (or tube) in which it originated (Tis, N0, M0).

Stage IA: The tumor is 2 cm or smaller in the pancreas. It has not spread to lymph nodes or other parts of the body (T1, N0, M0).

Stage IB: A tumor larger than 2 cm is in the pancreas. It has not spread to lymph nodes or other parts of the body (T2, N0, M0).

Stage IIA: A tumor extends beyond the pancreas, but the tumor has not spread to nearby arteries/veins. It has not spread to any lymph nodes or other parts of the body (T3, N0, M0).

Stage IIB: A tumor of any size has not spread to nearby arteries/veins. It has spread to lymph nodes but not to other parts of the body (T1, T2, or T3; N1; M0).

Stage III: A tumor has spread to nearby arteries, veins, and/or lymph nodes but has not spread to other parts of the body (T4, N1, M0).

Stage IV: Any tumor that has spread to other parts of the body (any T, any N, M1).

Recurrent: Recurrent cancer is cancer that comes back after treatment.

Used with permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Seventh Edition (2010)published by Springer-Verlag New York, www.cancerstaging.net.

Treatment

The treatment of pancreatic cancer depends on the size and location of the tumor, whether the cancer has spread, and the person’s overall health. In many cases, a team of doctors will 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.

When detected at an early stage, pancreatic cancer has a much higher chance of being successfully treated. However, there are treatments that can help control the disease even in patients with advanced pancreatic cancer that allows them to live longer.

The current treatment options for pancreatic cancer are surgery, radiation therapy, and/or chemotherapy. The specific treatment plan recommended depends on many factors. Descriptions of the most common treatment options for pancreatic cancer are listed below.

Surgery

Surgery may involve removing all or part of the pancreas, depending on the location and size of the cancer within the pancreas. A surgical oncologist is a doctor who specializes in treating cancer using surgery. If the cancer is still confined to the head of the pancreas, the surgeon may perform a Whipple procedure. This is an extensive operation where 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. An experienced surgeon should perform this procedure.

If the cancer is located in the tail of the pancreas, the common operation is a distal pancreatectomy, in which the surgeon removes the tail and body of the pancreas, as well as the spleen. If the cancer diffusely involves the pancreas, or is present at multiple sites within the pancreas, a total pancreatectomy may be required, where the surgeon removes the entire pancreas, part of the small intestine, a portion of the stomach, the common bile duct, the gallbladder, the spleen, and nearby lymph nodes. In all of the above surgeries, nearby lymph nodes are removed as part of the operation.

Sometimes before one of the above major operations, the surgeon may choose to start with a laparoscopy, in which several small holes are made in the abdomen, through which a tiny camera can be passed while a patient is under anesthesia. This allows the surgeon to determine whether the cancer has spread to other areas within the abdominal cavity, in which case undertaking the full operation to remove the primary tumor would not be appropriate.

In general, surgery may be combined with radiation therapy and/or chemotherapy. These may be given either before (neoadjuvant therapy) or after surgery (adjuvant therapy), although they are most often given afterwards. The purpose of giving radiation therapy and chemotherapy is to try to decrease the likelihood of the cancer returning. If it is unclear whether a cancer can be removed surgically (called borderline resectable) at the time of diagnosis, radiation therapy and/or chemotherapy may be given first to try and increase the chance of resection. Gemcitabine (Gemzar)-based chemotherapy is commonly given after surgery, based on evidence that it improves disease-free and overall survival. The role of radiation therapy after surgery remains somewhat controversial, although it is frequently used for individuals who have a high risk of their cancer coming back in the area of surgery (if it is a large tumor, or if there were close or positive surgical margins [area of tissue around the tumor site that contained cancer or may contain cancer]).

If the tumor is blocking the common bile duct or small intestine, placement of a stent (a tiny tube that helps keep the blocked area open and can be either metal or plastic) can be performed to relieve the blockage using nonsurgical approaches, such as ERCP, PTC, or endoscopy (see the Diagnosis section for more information). In some instances, the patient may need surgery to create a bypass, even if the tumor itself cannot be completely removed.

Side effects of surgery include weakness, tiredness, and pain after the first few days after the procedure. The doctor can prescribe medicine to provide relief. 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 medications may help. Also, the doctor can prescribe hormones and enzymes to replace those lost by the removal of the pancreas. Another common side effect is the development of diabetes due to the loss of insulin (produced by the pancreas), especially following a total pancreatectomy. In this situation, the doctor usually prescribes insulin. Learn more about cancer surgery.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. Radiation therapy is commonly given in pancreatic cancer in the following situations: after surgery (although this remains controversial, as mentioned above); before surgery to try and “downstage” a borderline resectable tumor; for patients with locally advanced disease; and occasionally in patients with metastatic disease, (for example, for relief of severe pain).

Newer radiation therapy techniques, such as stereotactic radiosurgery (for example, Cyberknife), are starting to play more of a role in the treatment of pancreatic cancer, with the advantage that they can provide more localized treatment and require only one or a small number of treatment sessions. However, these approaches have not been compared with the more conventional approach of delivering external-beam radiation therapy, which typically takes five to six weeks, and should not be considered a replacement for it.

Often, chemotherapy will be given simultaneously (at the same time) with radiation therapy because it can enhance the effects of the radiation therapy (called radiosensitization). However, chemotherapy given at the same time as radiation therapy generally has to be given at lower doses than when given alone.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. Learn more about radiation therapy.

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 is given by a medical oncologist, a doctor who specializes in treating cancer with medication. Some people may receive chemotherapy in their doctor's office; others may go to the hospital. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a specific time.

The most common first-line chemotherapy used for the treatment of pancreatic cancer is gemcitabine. Gemcitabine has been shown to improve survival outcomes in patients with advanced pancreatic cancer, as well as improve cancer-related symptoms (for example, weight loss, pain, and weakness) in some patients. A number of large studies have been conducted to evaluate whether combining gemcitabine with other drugs (such as fluorouracil [5-FU, Adrucil], capecitabine [Xeloda], or platinum compounds, including cisplatin [Platinol], and oxaliplatin [Eloxatin]) is more effective than gemcitabine alone. Some studies suggest modest improvements in outcomes using combination therapy; however, these combination treatments also tend to be associated with greater side effects. One large study demonstrated a modest survival benefit for patients with advanced pancreatic cancer receiving gemcitabine in combination with an oral medication called erlotinib (Tarceva) compared with gemcitabine alone. On this basis, erlotinib was approved by the U.S. Food and Drug Administration (FDA) in 2005 for use in advanced pancreatic cancer in combination with gemcitabine. Another large study conducted in Europe suggested a survival benefit using the combination of gemcitabine plus capecitabine (Xeloda), although final results of this study have not yet been published. Therefore, while gemcitabine alone has represented the standard of care for patients with advanced pancreatic cancer since its approval in 1997, this standard may be evolving as certain combination treatments demonstrate small but real advantages with manageable side effects for patients. These combination treatments are best for patients with good general strength and vigor.

Additionally, if a tumor continues to grow following first-line treatment with a gemcitabine-based therapy, there may still be a benefit from additional treatment using different drugs if the patient’s overall health remains strong. For instance, one recently reported study showed that the combination of 5-FU and oxaliplatin is effective in some patients as second-line treatment.

Side effects of chemotherapy depend on which drugs the patient receives. These include poor appetite, nausea, vomiting, diarrhea, mouth sores, hair loss, and a lack of energy. People undergoing chemotherapy also are more likely to get infections and bruise and bleed easily because chemotherapy decreases bone marrow production of white blood cells, red blood cells, and platelets. Certain drugs used in pancreatic cancer are also associated with specific side effects. Erlotinib, for example, frequently causes a skin rash similar to acne, and capecitabine can cause redness and discomfort on the palms of the hands and the soles of the feet. This condition is called hand-foot syndrome. These side effects typically go away between treatments and after the treatments have ended. The doctor can suggest ways to relieve these side effects.

Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

Advanced pancreatic cancer

In advanced disease, the cancer has metastasized beyond the pancreas to nearby lymph nodes and to distant organs. In patients where the cancer has spread to other organs away from the pancreas, chemotherapy alone is generally the treatment of choice. The patient may also consider enrolling in clinical trials of new treatments. Radiation Therapy or surgery are primarily for palliation (relief of symptoms to improve a person’s quality of life).

For patients with locally advanced disease, the combination of chemotherapy and radiation therapy may, in rare instances, shrink the tumor enough so it can be removed by surgery, referred to as downstaging (see Radiation Therapy section above).

Recurrent cancer

Pancreatic cancer that returns after treatment is known as recurrent cancer. The treatment options are generally the same as metastatic cancer, and usually center on chemotherapy, although surgery and radiation therapy may again be used for palliative purposes. Additionally, medication or a nerve block to reduce pain may be given. Clinical trials that test new therapies may also be available.

Find out more about common terms used during cancer treatment.

Clinical Trials Resources

Doctors and scientists are always looking for better ways to treat patients with pancreatic cancer. 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. The clinical trial may be evaluating a new drug, a new combination of existing treatments, a new approach to radiation therapy or surgery, or a new method of treatment or prevention. 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 pancreatic cancer. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with pancreatic cancer.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill”. The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

To join a clinical trial, patients must participate in a 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 than 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.

For specific topics being studied for pancreatic cancer, learn more in the Current Research section.

Side Effects

Cancer and its treatment can cause a variety of side effects. Abdominal and/or back pain are common side effects of pancreatic cancer. In addition to appropriate pain medications, a specialized type of nerve block (called a celiac plexus block, or celiac neurolysis) can often be performed by a pain specialist to relieve the pain associated with pancreatic cancer. Doctors have made major strides in recent years not only in treating pain but also in addressing nausea, vomiting, and other physical side effects associated with pancreatic cancer and cancer treatments. Many such treatments used today are less intensive but as effective as treatments used in the past.

Fear of treatment side effects is common after a diagnosis of cancer, 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 your doctor about side effects you experience during and after treatment. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them.

In addition to physical side effects, you may experience psychosocial (emotional and social) effects as well. Learn more about the importance of addressing such needs, including concerns about managing the cost of your cancer care.

Learn more about late effects or long-term side effects by reading the After Treatment section or talking with your doctor.

After Treatment

After treatment for pancreatic cancer 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.

For people who have had surgery, follow-up visits every three to six months with the oncologist are typically recommended. Blood tests, including monitoring of liver function tests and the tumor marker CA 19-9, can be checked during these visits. Routinely, CT scans do not need to be performed, but they may be appropriate depending on an individual’s symptoms and any abnormalities detected during the physical examination or with the blood work.

People recovering from pancreatic cancer 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.

Find out more about common terms used after cancer treatment is complete.

Current Research

Research for pancreatic cancer 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.

Early detection. Because the best chance of successful treatment occurs when pancreatic cancer is detected early, several efforts are ongoing to find and use specialized blood tests, diagnostic imaging tools, and other approaches to detect pancreatic cancer at its earliest stages, even at precancerous stages (known as pancreatic intraepithelial neoplasia, or PanIN lesions), before it has had the opportunity to spread. These screening efforts are typically being used for individuals at particularly high risk for pancreatic cancer, such as those with a strong family history or a known genetic predisposition to pancreatic cancer. It remains uncertain whether these tools will be useful for the general population.

Genetic studies. In cancer, damaged or abnormal genes cause uncontrolled cell growth. Many of the new developments are based on fixing or correcting damaged genes and proteins. Genetic studies are being done to find defective genes that are involved in pancreatic cancer. Once genes are identified, doctors can begin to screen people who may be at risk for pancreatic cancer.

Immunotherapy. Immunotherapy (also called biologic therapy) is designed to boost the body’s natural defenses to fight cancer. It uses materials either made by the body or in a laboratory to bolster, target, or restore immune system function. One example of immunotherapy is cancer vaccines, which stimulate a person’s immune system to recognize and attack cancer cells. A number of clinical trials have been done or are in progress studying vaccines in a variety of types of cancer, including pancreatic cancer. To date, however, there have been no vaccines with sufficient outcomes for them to become a standard treatment.

Targeted therapy.Targeted therapy is a treatment that targets faulty genes or proteins that contribute to cancer growth and development. Erlotinib is the only targeted therapy currently approved for pancreatic cancer, in combination with gemcitabine. Erlotinib is a small molecule inhibitor that disrupts signaling through a pathway called epidermal growth factor receptor (EGFR). This EGFR can be overactivated and lead to tumor cell growth. Other agents targeting different pathways important to tumor cell survival, division, and metastasis are under investigation in pancreatic cancer, both as single agents and as part of combination therapy. However, to date none (including bevacizumab [Avastin] and cetuximab [Erbitux]) have demonstrated survival benefit when evaluated in large phase III clinical trials.

Gene therapy. Gene therapy involves delivery of specific genes to cancer cells, which are often carried by specially designed viruses. These include normal genes that are delivered into the core of cancer cells; as the cancer cells divide, functional genes replace defective ones. Another strategy under investigation involves delivery of the gene TNF-alpha (an immune system protein) by direct injection into the tumor. This gene gets activated or “turned on” when radiation therapy is given, possibly enhancing anti-tumor activity.

Other chemotherapy agents. Several other chemotherapy agents have shown promise in advanced pancreatic cancer and have been studied in combination with gemcitabine. These include new formulations of paclitaxel (Taxol) that may improve drug delivery, such as nanoparticle albumin-bound paclitaxel (Abraxane), EndoTAG-1, and TS-1. These agents are currently undergoing testing in phase III clinical trials. TS-1 is an oral drug similar to capecitabine in how it works; it is approved for use in parts of Asia, but is not readily available in the United States.

Multidrug therapy. Multidrug or combination therapy combines existing drugs to fight pancreatic cancer; new combinations are under review. Researchers think that killing cancer cells with different kinds of drugs may be more effective than using a single drug.

The website www.cancermap.org is the National Cancer Institute’s Cancer Research Map, a listing of all public and privately funded research in pancreatic cancer.

Cancer stem cells.Pancreatic cancer stem cells represent a subset of cells within the tumor that may be particularly resistant to standard therapies. Research is currently focusing on identifying therapeutic agents that may specifically target those cancer stem cells.

Questions to Ask the Doctor

Regular communication with your doctor is important in making informed decisions about your health care. Consider asking the following questions of your doctor:

• What type of pancreatic cancer do I have?

• Is my cancer limited to the pancreas?

• What is the stage of the disease? What does this mean?

• Can you explain my pathology report (laboratory test results) to me?

• What are my treatment options?

• What clinical trials are open to me?

• What is the goal of each treatment? What is my prognosis (chance of recovery)?

• What treatment, or combination of treatments, do you recommend? Why?

• Is surgery a possibility to remove the cancer?

• How experienced is my surgeon in performing this type of operation?

• If surgery is not a possibility, what are my other options to treat my cancer?

• Can chemotherapy control my cancer?

• Should I receive radiation therapy? Why or why not?

• What are the side effects of this treatment, 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?

• If I’m worried about managing the costs related to my cancer care, who can help me with these concerns?

• What is my prognosis (chance of recovery)?

• What follow-up tests will I need, and how often will I need them?

• What support services are available to me? To my family?

Patient Information Resources

In addition to Cancer.Net, there are other sources of information about this type of cancer available online. Cancer.Net maintains a list of national, not-for-profit organizations that may be helpful in finding additional information, services, and support. As always, be sure to talk with your doctor about questions you may have about information you find about this disease.

View organizations that offer information on this specific type of cancer.

Islet Cell Tumor

Overview

Function of the pancreas

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 composed of two major components:

• The exocrine component, made up of ducts and acini (small sacs on the end of the ducts), produces enzymes (specialized proteins) that are released into the small intestine to help the body digest and break down food, particularly fats.

• 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 specific hormones, with the most important one being insulin, a substance that helps control the amount of sugar in the blood.


Types of pancreatic cancer

Cancer begins when normal cells begin to change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

The most common type of pancreatic cancer is called ductal adenocarcinoma, or simply, adenocarcinoma. In this type, the cancer begins in the exocrine component. Learn more about adenocarcinoma of the pancreas in the Guide to Pancreatic Cancer.

Much less commonly, tumors can begin in the islets of Langerhans, the endocrine component. An islet cell tumor can also be called a pancreatic islet cell tumor, pancreatic endocrine tumor, Islet of Langerhans tumor, or neuroendocrine tumor. The rest of this section focuses on islet cell tumors.

Subtypes of islet cell tumors

An islet cell tumor can be either benign or cancerous. Also, an islet cell tumor may be classified as either functioning or nonfunctioning. The term functioning means that the tumor cells produce hormones that cause specific symptoms, while a nonfunctioning islet cell tumor does not produce hormones.

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 symptoms or syndromes described above. As a result, they are typically diagnosed at more advanced stages of disease.

Find out more about basic terms used in this section.

Statistics

Islet cell tumors are uncommon, with 200 to 1,000 new cases diagnosed each year in the United States. They are often treatable.

Cancer 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.

Source: National Cancer Institute

Risk Factors

A risk factor is anything that increases a person’s chance of developing a tumor. 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 a tumor, most do not directly cause a tumor. Some people with several risk factors never develop a tumor, 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. The following factor may raise a person’s risk of developing an islet cell tumor:

MEN1. 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 Guide to Multiple Endocrine Neoplasia, Type 1.

Symptoms

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 a tumor. If you are concerned about a symptom on this list, please talk with your doctor.

Gastrinoma

• Ulcers (as a result of increased stomach acid)

• Diarrhea


Insulinoma

• Hypoglycemia, which causes fatigue, 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 (can lead to irregular heartbeat, muscle cramping and weakness, and decreased reflexes)

• Low level of hydrochloric acid in the stomach (can lead to digestive problems and improper absorption of vitamins and nutrients)

• 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 (can lead to digestive problems and improper absorption of vitamins and nutrients)


Other symptoms

• Abdominal pain

• Jaundice (yellowing of the skin and whites of the eyes)

• Vomiting blood

• Sweating

• Rapid heart rate

• Anxiety

• Headache

• Convulsions

• Loss of consciousness

• Clouding of vision

• Unexplained weight gain or loss

• Inflamed mouth and tongue

• A mass in the abdomen


Diagnosis

Doctors use many tests to diagnose a tumor and determine if it is cancerous, and if so, 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. There are different types of ultrasounds. 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 sometimes best shown using an MRI, especially if they have spread to the liver.

Octreotide scan. An octreotide scan is a special type of nuclear medicine scan used to detect the location and possible spread of an islet cell tumor. A person is given a small amount of a radioactive substance, which shows up on pictures taken by a gamma camera. The test takes place over several days.

Learn more about what to expect when having common tests, procedures, and scans.

Find out more about common terms used during a diagnosis of cancer.

Staging

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


Treatment

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.

Descriptions of the most common treatment options for an islet cell tumor are listed below.

Active surveillance

Sometimes, an active surveillance (also called watchful waiting or watch-and-wait) approach 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. A surgical oncologist is a doctor who specializes in treating cancer using surgery.

Common surgical procedures that may be used in treating a person with an islet cell tumor include:

• Enucleation. In this surgery, only the tumor itself is removed.

• Whipple procedure. During this 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.

• Distal pancreatectomy. If the cancer is located in the tail of the pancreas, the common operation is a distal pancreatectomy, in which the surgeon removes the tail and body of the pancreas, as well as the spleen.

• Splenectomy. Spleen removal is called a splenectomy.

• Gastrectomy. For a gastrinoma, it may also be necessary to take out the stomach to remove ulcers, a procedure called a gastrectomy.


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. Learn more about cancer surgery.

If all or part of the pancreas was removed during surgery, it may be difficult to digest food. 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 of pancreas removal 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 is given by a medical oncologist, a doctor who specializes in treating cancer with medication. Some people may receive chemotherapy in their doctor's office; others may go to the hospital. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a specific time. 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.

Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using 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. However, it can have significant side effects including fatigue, depression, and flu-like symptoms. Learn more about immunotherapy.

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. Sometimes this procedure also involves delivery of chemotherapy directly into the tumor prior to blocking the blood supply to that portion of the liver (called chemoembolization, or transarterial chemoembolization [TACE]), or delivery of radioactively labeled microbeads (called radioembolization, such as TheraSpheres or SIR-Spheres).

Find out more about common terms used during treatment.

Clinical Trials Resources

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. The clinical trial may be evaluating a new drug, a new combination of existing treatments, a new approach to radiation therapy or surgery, or a new method of treatment or prevention. 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.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill”. The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

To join a clinical trial, patients must participate in a 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.

For specific topics being studied for an islet cell tumor, learn more in the Current Research section.

Side Effects

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 tumor, 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. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them.

In addition to physical side effects, you may experience psychosocial (emotional and social) effects as well. Learn more about the importance of addressing such needs, including concerns about managing the cost of your cancer care.

Learn more about late effects or long-term side effects by reading the After Treatment section or talking with your doctor.

After Treatment

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.

Find out more about common terms used after cancer treatment is complete.

Current Research

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, octreotide, and Everolimus (RAD001, see below) for patients with advanced islet cell tumors (see drug combinations and new agents below).

Other oral medications that have antiangiogenic properties and are also being studied in people with advanced islet cell tumors include sunitinib (Sutent), sorafenib (Nexavar), pazopanib, and AMG706. One large phase III study has recently shown that sunitinib significantly delays the growth and spread of islet cell tumors.

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. RAD001, a new drug that works by inhibiting a protein important in cancer cell growth called mTOR, 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.

Questions to Ask the Doctor

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 (laboratory test results) 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?

• If I’m worried about managing the costs related to my cancer care, who can help me with these concerns?


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?


Patient Information Resources

In addition to Cancer.Net, there are other sources of information about this type of cancer available online. Cancer.Net maintains a list of national, not-for-profit organizations that may be helpful in finding additional information, services, and support. As always, be sure to talk with your doctor about questions you may have about information you find about this disease.

View organizations that offer information on this specific type of cancer.