Gastrointestinal Carcinoid Tumors

 

 

A neuroendocrine carcinoid tumor is cancer that forms in the lining of the gastrointestinal tract.

 

The gastrointestinal tract includes the stomach, small intestine, and large intestine. These organs are part of the digestive system, which processes nutrients ( vitamins, minerals, carbohydrates, fats, proteins, and water) in foods that are eaten and helps pass waste material out of the body. Neuroendocrine carcinoid tumors develop from a certain type of hormone -making cell in the lining of the gastrointestinal tract. These cells produce hormones that help regulate digestive juices and the muscles used in moving food through the stomach and intestines. A neuroendocrine carcinoid tumor may also produce hormones. Carcinoid tumors that start in the rectum (the last several inches of the large intestine) usually do not produce hormones.

 

Neuroendocrine carcinoid tumors grow slowly. Most of them occur in the appendix (an organ attached to the large intestine), small intestine, and rectum. It is common for more than one tumor to develop in the small intestine. Having a carcinoid tumor increases a person's chance of getting other cancers in the digestive system, either at the same time or later.

 

Health history can affect the risk of developing neuroendocrine carcinoid tumors.

 

Risk factors include the following:

 

• Having a family history of multiple endocrine neoplasia type 1 (MEN1) syndrome.

• Having certain conditions that affect the stomach's ability to produce stomach acid, such as atrophic gastritis, pernicious anemia, or Zollinger-Ellison syndrome.

• Smoking tobacco.

 

A neuroendocrine carcinoid tumor often has no signs in its early stages. Carcinoid syndrome may occur if the tumor spreads to the liver or other parts of the body.

 

The hormones produced by neuroendocrine carcinoid tumors are usually destroyed by blood and liver enzymes. If the tumor has spread to the liver, however, high amounts of these hormones may remain in the body and cause the following group of symptoms, called carcinoid syndrome:

 

• Redness or a feeling of warmth in the face and neck.

• Diarrhea.

• Shortness of breath, fast heartbeat, tiredness, or swelling of the feet and ankles.

• Wheezing.

• Pain or a feeling of fullness in the abdomen.

 

These symptoms and others may be caused by neuroendocrine carcinoid tumors or by other conditions. A doctor should be consulted if any of these symptoms occur.

 

Tests that examine the blood and urine are used to detect (find) and diagnose neuroendocrine carcinoid tumors.

 

The following tests and procedures may be used:

 

• Complete blood count: A procedure in which a sample of blood is drawn and checked for the following:

  • The number of red blood cells, white blood cells, and platelets.

  • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.

  • The portion of the sample made up of red blood cells.

• Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.

• Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances, such as hormones, released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that produces it. The blood sample is checked to see if it contains a hormone produced by carcinoid tumors. This test is used to help diagnose carcinoid syndrome.

• Twenty-four-hour urine test: A test in which a urine sample is checked to measure the amounts of certain substances, such as hormones. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that produces it. The urine sample is checked to see if it contains a hormone produced by carcinoid tumors. This test is used to help diagnose carcinoid syndrome.

 

Certain factors affect prognosis (chance of recovery) and treatment options.

 

The prognosis (chance of recovery) and treatment options depend on the following:

 

• Whether the cancer can be completely removed by surgery.

• Whether the cancer has spread from the stomach and intestines to other parts of the body, such as the liver or lymph nodes.

• The size of the tumor.

• Where the tumor is in the gastrointestinal tract.

• Whether the cancer is newly diagnosed or has recurred.

 

Treatment options also depend on whether the cancer is causing symptoms. Most neuroendocrine carcinoid tumors are slow-growing and can be treated and often cured. Even when not cured, many patients may live for a long time.

 

Treatment Option Overview

 

Different types of treatment are available for patients with neuroendocrine carcinoid tumors. 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.

 

Seven types of standard treatment are used:

 

Surgery

 

Treatment of neuroendocrine carcinoid tumors usually includes surgery. One of the following surgical procedures may be used:

 

• Appendectomy: Removal of the appendix.

• Fulguration: Use of an electric current to burn away the tumor using a special tool.

• Cryosurgery: A treatment that uses an instrument to freeze and destroy abnormal tissue, such as carcinoma in situ. This type of treatment is also called cryotherapy. The doctor may use ultrasound to guide the instrument.

• Resection: Surgery to remove part or all of the organ that contains cancer. Resection of the tumor and a small amount of normal tissue around it is called a local excision.

• Bowel resection and anastomosis: Removal of the bowel tumor and a small section of healthy bowel on each side. The healthy parts of the bowel are then sewn together (anastomosis). Lymph nodes are removed and checked by a pathologist to see if they contain cancer.

• Radiofrequency ablation: The use of a special probe with tiny electrodes that release high-energy radio waves (similar to microwaves) that kill cancer cells. The probe may be inserted through the skin or through an incision (cut) in the abdomen.

• Hepatic resection: Surgery to remove part or all of the liver.

• Hepatic artery ligation or embolization: A procedure to ligate (tie off) or embolize (block) the hepatic artery, the main blood vessel that brings blood into the liver. Blocking the flow of blood to the liver helps kill cancer cells growing there.

 

 

Radiation therapy

 

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

 

Chemotherapy

 

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells 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 spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy).

 

Chemoembolization of the hepatic artery is a type of regional chemotherapy that may be used to treat a neuroendocrine carcinoid tumor that has spread to the liver. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with a substance that embolizes (blocks) the artery, cutting 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. 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.

 

The way the chemotherapy is given depends on the type and stage of the cancer being treated.

 

Percutaneous ethanol injection

Percutaneous ethanol injection is a cancer treatment in which a small needle is used to inject ethanol (alcohol) directly into a tumor to kill cancer cells. This procedure is also called intratumoral ethanol injection.

 

Biologic therapy

Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.

 

Hormone therapy

Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances produced by glands in the body and circulated in the bloodstream. The presence of 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 are used to reduce the production of hormones or block them from working.

 

Other drug therapy

MIBG (metaiodobenzylguanidine) is sometimes used, with or without radioactive iodine (I131), to lessen the symptoms of neuroendocrine carcinoid tumors.

 

New types of treatment are being tested in clinical trials.

 

Treatments being studied in clinical trials for neuroendocrine carcinoid tumors include new combinations of chemotherapy. 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 clinical trials database.

 

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.

 

Treatment Options by Tumor Type

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.

 

Localized Neuroendocrine Carcinoid Tumors

 

Carcinoid tumors in the appendix

Treatment of localized neuroendocrine carcinoid tumors in the appendix may include the following:

 

• Appendectomy.

• Appendectomy and local excision.

• Appendectomy, bowel resection with anastomosis, and removal of lymph nodes.

 

Rectal carcinoid tumors

Treatment of localized neuroendocrine carcinoid tumors in the rectum may include the following:

 

• Fulguration.

• Local excision.

• Resection.

 

Surgery that saves the sphincter muscles (the muscles that open and close the anus) may be possible.

 

Small bowel carcinoid tumors

Treatment of localized neuroendocrine carcinoid tumors in the small intestine may include the following:

• Local excision.

• Resection with removal of nearby lymph nodes.

 

Gastric, colon, and pancreatic carcinoid tumors

Treatment of localized neuroendocrine carcinoid tumors in the stomach, colon, or pancreas is usually resection.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with localized neuroendocrine carcinoid 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.

 

Regional Neuroendocrine Carcinoid Tumors

Treatment is usually surgery to remove all the cancer that can be seen at the site of the original tumor, as well as nearby tissues and lymph nodes.

 

If the tumor cannot be completely removed by surgery, treatment is usually palliative therapy to relieve symptoms and improve the patient's quality of life. This may include the following:

• Resection, cryosurgery, or radiofrequency ablation to remove as much of the tumor as possible.

• Chemoembolization to shrink tumors in the liver.

 

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with regional neuroendocrine carcinoid 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.

 

Metastatic Neuroendocrine Carcinoid Tumors

 

Distant metastases

If the metastatic neuroendocrine carcinoid tumor is not causing symptoms, there may be a period of watchful waiting before treatment is given. Treatment of distant metastases of neuroendocrine carcinoid tumors is usually palliative therapy that may include the following:

 

• Surgery to bypass or remove part of a tumor blocking the small intestine.

• Chemotherapy, which may include chemoembolization.

• Radiation therapy, sometimes with radioisotopes such as radioactive iodine (I131).

• MIBG (metaiodobenzylguanidine) therapy.

• Biologic therapy and/or hormone therapy.

• Clinical trials of new treatments.

 

Carcinoid syndrome

Treatment of metastatic neuroendocrine carcinoid tumors that are causing carcinoid syndrome may include the following:

 

• Resection, cryosurgery, radiofrequency ablation, or percutaneous ethanol injection for tumors in the liver.

• Hepatic artery ligation or embolization, with or without regional or systemic chemotherapy.

• Hormone therapy.

• Biologic therapy with or without chemotherapy.

• Clinical trials of new combinations of chemotherapy.

 

A heart valve replacement may be done for some patients with carcinoid syndrome.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with metastatic neuroendocrine carcinoid 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 Neuroendocrine Carcinoid Tumors

Treatment of recurrent neuroendocrine carcinoid tumors may include the following:

 

• Surgery to remove part or all of the tumor.

• A clinical trial.

 

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent neuroendocrine carcinoid 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 Neuroendocrine Tumor

A recurrent neuroendocrine carcinoid tumor is a tumor that has recurred (come back) after it has been treated. The tumor may come back in the stomach or intestines or in other parts of the body.

Treatment of recurrent neuroendocrine carcinoid tumors may include the following:

 

• Surgery to remove part or all of the tumor.

• A clinical trial.

 

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent neuroendocrine carcinoid 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.