患者さん向け Childhood Gastrointestinal Carcinoid Tumors Treatment (PDQ®)

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This PDQ cancer information summary has current information about the treatment of childhood gastrointestinal carcinoid tumors. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change. The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.

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General Information About Childhood Gastrointestinal Carcinoid Tumors

A gastrointestinal carcinoid tumor forms in neuroendocrine cells in the lining of the digestive tract, appendix, and other organs in the abdomen.

Gastrointestinal carcinoid tumors form from a certain type of neuroendocrine cell (a type of cell that is like a nerve cell and a hormone-making cell). These cells are scattered throughout the chest and abdomen, but most are found in certain organs in the abdomen. Neuroendocrine cells in the digestive tract make hormones that help control digestive juices and the muscles used in moving food through the stomach and intestines.

Most gastrointestinal carcinoid tumors in children form in the appendix (a pouch that sticks out from the first part of the large intestine near the end of the small intestine). The tumor is often found during surgery to remove the appendix. They also form in the digestive tract (lining of the stomach or intestines), pancreas, and liver.

These tumors are usually small, slow-growing, and benign (not cancer). Some tumors may be malignant (cancer) and spread to other places in the body.

Signs and symptoms of gastrointestinal carcinoid tumors depend on where the tumor forms.

Check with your child's doctor if you are concerned about signs and symptoms that may be caused by gastrointestinal carcinoid tumors or by other conditions.

Carcinoid tumors in the appendix may cause the following signs and symptoms:

Gastrointestinal carcinoid tumors that are not in the appendix may release hormones and other substances. Carcinoid syndrome occurs when a carcinoid tumor in the digestive tract releases the hormone serotonin and other substances. It may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

Tests that examine the digestive tract, liver, and pancreas are used to help diagnose gastrointestinal carcinoid tumors.

The following tests and procedures may be used:

Certain factors affect prognosis (chance of recovery).

Prognosis depends on the following:

The prognosis for carcinoid tumors in the appendix in children is usually excellent after surgery to remove the tumor. Gastrointestinal carcinoid tumors that are not in the appendix are usually larger or have spread to other parts of the body at the time of diagnosis and do not respond well to chemotherapy. Larger tumors are more likely to recur (come back).

Stages of Gastrointestinal Carcinoid Tumors

After a gastrointestinal carcinoid tumor has been diagnosed, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.

The process used to find out if a gastrointestinal carcinoid tumor has spread to nearby areas or other parts of the body is called staging. Carcinoid tumors of the appendix are not known to spread, but other gastrointestinal carcinoid tumors may spread. There is no standard staging system for childhood gastrointestinal carcinoid tumors. The results of tests and procedures done to diagnose gastrointestinal carcinoid tumors are used to help make decisions about treatment.

Sometimes childhood gastrointestinal carcinoid tumors recur (come back) after treatment.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if carcinoid tumor cells in the small intestine spread to the liver, the cancer cells in the liver are actually carcinoid tumor cells. The cells in the liver are metastatic carcinoid tumor cells, not liver cancer.

Treatment Option Overview

There are different types of treatment for children with gastrointestinal 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.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with gastrointestinal carcinoid tumors not in the appendix should have their treatment planned by a team of doctors who are experts in treating childhood cancer.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:

Five types of standard treatment are used:

Surgery

Surgery to remove the tumor is the only treatment needed for carcinoid tumors in the appendix.

Embolization

Embolization is a treatment in which contrast dye and particles are injected into the hepatic artery through a catheter (thin tube). The particles block the artery, cutting off blood flow to the tumor. Sometimes a small amount of a radioactive substance is attached to the particles. Most of the radiation is trapped near the tumor to kill the cancer cells. This is called radioembolization.

Hormone therapy

Hormone therapy with a somatostatin analogue (octreotide or lanreotide) may be used to treat gastrointestinal carcinoid tumors that have spread or cannot be removed by surgery. This treatment stops extra hormones from being made by the carcinoid tumor. Octreotide or lanreotide are somatostatin analogues which are injected under the skin or into the muscle.

Peptide receptor radionuclide therapy

Sometimes a small amount of a radioactive substance is attached to the somatostatin analogue drugs octreotide or lanreotide to kill the cancer cells.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment of gastrointestinal carcinoid tumors may cause side effects.

For information about side effects that begin during treatment for cancer, see our Side Effects page.

Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.

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. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

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.

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 child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment of Gastrointestinal Carcinoid Tumors

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of newly diagnosed carcinoid tumors in the appendix in children may include the following:

Treatment of newly diagnosed carcinoid tumors in the large intestine, pancreas, or stomach is usually surgery.

Treatment of newly diagnosed tumors that cannot be removed by surgery, multiple tumors, or tumors that have spread may include the following:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Recurrent Gastrointestinal Carcinoid Tumors

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of recurrent gastrointestinal carcinoid tumors in children may include the following:

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

To Learn More About Gastrointestinal Carcinoid Tumors

For more information from the National Cancer Institute about gastrointestinal carcinoid tumors, see the following:

For more childhood cancer information and other general cancer resources, see the following:

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of childhood gastrointestinal carcinoid tumors. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." 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.

Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).

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PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Gastrointestinal Carcinoid Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/gi-carcinoid-tumors/patient/child-gi-carcinoid-treatment-pdq. Accessed <MM/DD/YYYY>.

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