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Pancreatic neuroendocrine tumors form in hormone-making cells (islet cells) of the pancreas.
The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine. Anatomy of the pancreas. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs.
There are two kinds of cells in the pancreas:
A pancreatic neuroendocrine tumor (NET) may also be called a pancreatic endocrine tumor (PET), islet cell tumor, islet cell carcinoma, or pancreatic carcinoid.
Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis.
Pancreatic NETs may or may not cause symptoms.
Pancreatic NETs may be functional (the hormones that are released cause symptoms) or nonfunctional (the hormones that are released do not cause symptoms) tumors:
Most pancreatic NETs are functional tumors.
There are different kinds of functional pancreatic NETs.
Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following:
Having certain syndromes can increase the risk of pancreatic NETs.
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk.
Multiple endocrine neoplasia type 1 (MEN1) syndrome is a risk factor for pancreatic NETs.
Different types of pancreatic NETs have different signs and symptoms.
Symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes. Some tumors may not cause symptoms. Conditions other than pancreatic NETs can cause the symptoms listed below. Check with your doctor if you have any of these problems.
Signs and symptoms of a non-functional pancreatic NET
A non-functional pancreatic NET may grow for a long time without causing symptoms. It may grow large or spread to other parts of the body before it causes symptoms, such as:
Signs and symptoms of a functional pancreatic NET
The symptoms of a functional pancreatic NET depend on the type of hormone being made.
Too much gastrin may cause:
Too much insulin may cause:
Too much glucagon may cause:
Too much vasoactive intestinal peptide (VIP) may cause:
Too much somatostatin may cause:
Lab tests and imaging tests are used to detect (find) and diagnose pancreatic NETs.
The following tests and procedures may be used:
Other kinds of lab tests are used to check for the specific type of pancreatic NETs.
Other tumor types
Certain factors affect prognosis (chance of recovery) and treatment options.
Pancreatic NETs can often be cured. The prognosis (chance of recovery) and treatment options depend on the following:
The plan for cancer treatment depends on where the NET is found in the pancreas and whether it has spread.
The process used to find out if cancer has spread within the pancreas or to other parts of the body is called staging. The results of the tests and procedures used to diagnose pancreatic neuroendocrine tumors (NETs) are also used to find out whether the cancer has spread. See the General Information section for a description of these tests and procedures.
Although there is a standard staging system for pancreatic NETs, it is not used to plan treatment. Treatment of pancreatic NETs is based on the following:
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
Recurrent pancreatic neuroendocrine tumors (NETs) are tumors that have recurred (come back) after being treated. The tumors may come back in the pancreas or in other parts of the body.
There are different types of treatment for patients with pancreatic NETs.
Different types of treatments are available for patients with pancreatic neuroendocrine tumors (NETs). Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Six types of standard treatment are used:
An operation may be done to remove the tumor. One of the following types of surgery may be used:
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated.
Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working.
Hepatic arterial occlusion or chemoembolization
Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery (the major blood vessel that carries blood to the liver). This is done to kill cancer cells growing in the liver. The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine.
Chemotherapy delivered during hepatic arterial occlusion is called chemoembolization. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with the substance that blocks the artery and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body.
The blockage may be temporary or permanent, depending on the substance used to block the artery.
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Certain types of targeted therapies are being studied in the treatment of pancreatic NETs.
Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for pancreatic NETs may include treatment for the following:
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI Web site.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Treatment of gastrinoma may include supportive care and the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with gastrinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Treatment of insulinoma may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with insulinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Treatment may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with glucagonoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Other Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)
For VIPoma, treatment may include the following:
For somatostatinoma, treatment may include the following:
Treatment of other types of pancreatic neuroendocrine tumors (NETs) may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with islet cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Recurrent or Progressive Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)
Treatment of pancreatic neuroendocrine tumors (NETs) that continue to grow during treatment or recur (come back) may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent islet cell carcinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
For more information from the National Cancer Institute about pancreatic neuroendocrine tumors (NETs), see the following:
For general cancer information and other resources from the National Cancer Institute, see the following:
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Last Revised: 2013-01-18
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