Childhood Bladder Cancer

Childhood Bladder Cancer

Childhood bladder cancer is a very rare type of cancer that forms in the tissues of the bladder.

The bladder is a hollow, balloon-shaped organ in the lower part of the abdomen that stores urine. The bladder has a muscular wall that allows it to get larger to store urine made by the kidneys, and to shrink to squeeze urine out of the body. There are two kidneys, one on each side of the backbone, above the waist. The bladder and kidneys work together to remove toxins and wastes from the body through urine:

  • Tiny tubules in the kidneys filter and clean the blood.
  • These tubules take out waste products and make urine.
  • The urine passes from each kidney through a long tube called a ureter into the bladder.
  • The bladder holds the urine until it passes through the urethra and leaves the body.
EnlargeAnatomy of the urinary system; drawing showing the right and left kidneys, the ureters, the bladder filled with urine, and the urethra. The inside of the left kidney shows the renal pelvis. An inset shows the renal tubules and urine. The spine and adrenal glands are also shown.
Anatomy of the urinary system showing the kidneys, ureters, bladder, and urethra. The inside of the left kidney shows the renal pelvis. An inset shows the renal tubules and urine. Also shown is the spine and adrenal glands. Urine is made in the renal tubules and collects in the renal pelvis of each kidney. The urine flows from the kidneys through the ureters to the bladder. The urine is stored in the bladder until it leaves the body through the urethra.

Urothelial carcinoma (also called transitional cell carcinoma) is cancer that begins in the urothelial cells, which line the urethra, bladder, ureters, renal pelvis, and some other organs. The cells of the urothelium are known as transitional cells because they are able to stretch when the bladder is full of urine and shrink when it is emptied. Urothelial carcinoma is the most common form of bladder cancer in children.

Squamous cell and other more aggressive types of bladder cancer are less common in children.

Bladder cancer can occur at any age and most often affects males.

Risk factors for childhood bladder cancer

Bladder cancer is caused by certain changes to the way bladder cells function, especially how they grow and divide into new cells. Learn more about how cancer develops at What Is Cancer?

The risk of bladder cancer is increased in children who have been treated for cancer with certain anticancer drugs, called alkylating agents, which include cyclophosphamide, ifosfamide, busulfan, and temozolomide. Survivors of heritable retinoblastoma also have an increased risk of developing bladder cancer. Not every child with these risk factors will develop bladder cancer. And it will develop in some children who don’t have a known risk factor. Talk with your child’s doctor if you’re concerned about your child’s risk.

Symptoms of childhood bladder cancer

The symptoms of bladder cancer can vary from person to person. The most common symptom of bladder cancer is blood in the urine, called hematuria. It’s often slightly rusty to bright red in color. You may see blood in your child’s urine at one point, then not see it again for a while. Sometimes there are very small amounts of blood in the urine that can only be found by having a test done.

Other common symptoms of bladder cancer can include:

  • frequent urination or feeling the need to urinate without being able to do so
  • pain during urination
  • abdominal or lower back pain

It’s important to check with your child’s doctor if your child has any of these symptoms. Keep in mind that urinary tract infections, kidney or bladder stones, or other problems related to the kidney could also be the cause, not cancer. Your child’s doctor will ask you when the symptoms started and how often your child has been having them. They’ll most likely ask for a urine sample as a first step in diagnosing what is causing these symptoms.

Tests to diagnose childhood bladder cancer

If your child has symptoms or lab test results that suggest bladder cancer, their doctor will need to find out if these are due to cancer or another problem. They may:

  • ask about your child’s personal and family medical history to learn more about your child’s symptoms and possible risk factors for bladder cancer
  • do a physical exam to check for signs of cancer
  • ask for a sample of urine so it can be checked in the lab for blood, abnormal cells, or infection

Depending on your child’s symptoms and medical history and the results of their physical exam and urine lab tests, the doctor may recommend more tests to find out if your child has bladder cancer, and if so, its extent (stage). The results of tests and procedures done to diagnose bladder cancer are used to help make decisions about treatment.

The following tests and procedures may be used:

CT scan (CAT scan)

A CT scan uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, such as the pelvis. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Learn more about Computed Tomography (CT) Scans and Cancer.

EnlargeComputed tomography (CT) scan; drawing shows a child lying on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
Computed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.

Ultrasound exam

An ultrasound exam uses high-energy sound waves (ultrasound) that bounce off internal tissues or organs, such as the pelvis, and make echoes. The echoes form a picture of body tissues called a sonogram.

EnlargeAbdominal ultrasound; drawing shows a child lying on an exam table during an abdominal ultrasound procedure. A technician is shown pressing a transducer (a device that makes sound waves that bounce off tissues inside the body) against the skin of the abdomen. A computer screen shows a sonogram (picture).
Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).

Cystoscopy

Cystoscopy is a procedure in which the doctor looks inside the bladder and urethra (the tube that carries urine out of your body) to check for abnormal areas. A cystoscope is slowly inserted through the urethra into the bladder to allow the doctor to see inside. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove very small tumors or tissue samples for biopsy. If a cystoscopy is not done at diagnosis, tissue samples are removed and checked for cancer during surgery to remove all or part of the bladder.

Getting a second opinion

You may want to get a second opinion to confirm your child’s bladder cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. They may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child’s tumor.

To learn more about choosing a doctor and getting a second opinion, see Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your child’s appointments, see Questions to Ask Your Doctor about Cancer.

Prognosis of childhood bladder cancer

If your child has been diagnosed with bladder cancer, you likely have questions about how serious the cancer is and your child’s chances of survival. The likely outcome or course of a disease is called prognosis.

The prognosis can be affected by whether the cancer can be removed by surgery. In children, bladder cancer is usually low grade (not likely to spread) and the prognosis is usually excellent after surgery to remove the tumor.

Types of treatment for childhood bladder cancer

There are different types of treatment for children and adolescents with bladder cancer. You and your child’s cancer care team will work together to decide treatment. Many factors will be considered, such as your child’s overall health and whether the cancer is newly diagnosed or has come back.

A pediatric oncologist, a doctor who specializes in treating children with cancer, will oversee treatment for childhood bladder cancer. The pediatric oncologist works with other health care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. Other specialists may include:

Your child’s treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s cancer care team before treatment begins about what to expect. For help every step of the way, see our downloadable booklet, Children with Cancer: A Guide for Parents.

Types of treatment your child might have include:

Surgery

Surgery to remove the cancer and part or all of the bladder is the standard treatment for bladder cancer in children. The type of surgery depends on where the cancer is located and whether it is aggressive.

Talk with your child’s doctor about how surgery for bladder cancer can affect urinating, sexual function, and fertility. Learn more about Fertility Issues in Girls and Women with Cancer and Fertility Issues in Boys and Men with Cancer.

Transurethral resection (TUR)

During TUR, the doctor removes tissue from the bladder using a resectoscope inserted into the bladder through the urethra. A resectoscope is a thin, tube-like instrument with a light, a lens for viewing, and a tool to remove tissue and burn away any remaining tumor cells. Tissue samples from the area where the tumor was removed are checked under a microscope for signs of cancer.

Cystectomy

Cystectomy, surgery to remove part or all of the bladder, is rarely used to treat bladder cancer in children. However, it may be needed in children with squamous cell carcinoma or more aggressive carcinomas.

Clinical trials

For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.

You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child’s age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.

Treatment of childhood bladder cancer

Treatment of newly diagnosed bladder cancer in children is usually surgery:

  • transurethral resection (TUR)
  • surgery to remove the bladder (rare)

Learn more about these treatments in the Types of treatment section.

Sometimes bladder cancer can recur (come back) after treatment. If your child is diagnosed with recurrent bladder cancer, your child’s doctor will work with you to plan treatment.

Side effects and late effects of cancer treatment

Learn more about Side Effects of Cancer Treatment.

Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:

  • physical problems
  • changes in mood, feelings, thinking, learning, or memory
  • second cancers (new types of cancer) or other conditions

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. Learn more about Late Effects of Treatment for Childhood Cancer.

Follow-up care

Some of the tests that were done to diagnose the cancer may be repeated to see how well the treatment worked.

If the bladder cancer recurs (comes back) after treatment, it will usually be within 3 years from diagnosis. Your child will receive tests from time to time after surgery to find out if their condition has changed or if the cancer has recurred. These tests are sometimes called follow-up tests or check-ups.

Coping and support

When a child has cancer, every member of the family needs support. Taking care of yourself during this difficult time is also important. Reach out to your child’s treatment team and to people in your family and community for support. To learn more, see Support for Families: Childhood Cancer.

Childhood Gastrointestinal Stromal Tumors Treatment (PDQ®)–Patient Version

Childhood Gastrointestinal Stromal Tumors Treatment (PDQ®)–Patient Version

General Information About Childhood Gastrointestinal Stromal Tumors (GIST)

Key Points

  • Gastrointestinal stromal tumors are a disease in which malignant (cancer) cells form in the tissues of the wall of the stomach or intestines.
  • GIST may develop as part of a syndrome.
  • Signs and symptoms of GIST include anemia and a lump in the abdomen.
  • Tests that examine the digestive tract are used to help diagnose GIST.

Gastrointestinal stromal tumors are a disease in which malignant (cancer) cells form in the tissues of the wall of the stomach or intestines.

Gastrointestinal stromal tumors (GIST) usually begin in cells in the tissues of the wall of the stomach or intestines. These cells, called interstitial cells of Cajal, help food move along the digestive tract.

Childhood GIST usually occurs in the stomach. It is more common in girls, and typically appears in the teen years.

GIST in children is not the same as GIST in adults. Patients should be seen at centers that specialize in the treatment of GIST.

EnlargeDrawing of the gastrointestinal tract showing the stomach, small intestine, colon, and rectum.
Gastrointestinal stromal tumors (GISTs) are most common in the stomach and small intestine but may be found anywhere in or near the gastrointestinal tract.

GIST may develop as part of a syndrome.

GIST may occur as part of the following syndromes:

Signs and symptoms of GIST include anemia and a lump in the abdomen.

These and other signs and symptoms may be caused by GIST or by other conditions.

Check with your child’s doctor if your child has any of the following:

  • Anemia (tiredness, dizziness, fast or irregular heartbeat, shortness of breath, or pale skin).
  • A lump in the abdomen.
  • A blockage of the intestine (cramping pain in the abdomen, nausea, vomiting, diarrhea, constipation, and swelling of the abdomen).

Tests that examine the digestive tract are used to help diagnose GIST.

The following tests and procedures may be used:

  • Physical exam and health 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.
  • Gene testing: A laboratory test in which cells or tissue are analyzed to look for changes in the KIT, PDGFA, and SDH genes. It is important to know whether there are changes in these genes in order to diagnose GIST and plan treatment.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
    EnlargePositron emission tomography (PET) scan; drawing shows a child lying on table that slides through the PET scanner.
    Positron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child’s vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
  • X-ray: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body, such as the abdomen or the area where the tumor formed.
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
    • Fine-needle aspiration: The removal of tissue using a thin needle.
    • Endoscopy: A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth or anus. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease.

    The following laboratory test may be done to study the tissue samples:

    • Immunohistochemistry: A laboratory test that uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test looks for the enzyme SDH in a sample of the patient’s tissue. When SDH is not present, it is called SDH-deficient GIST. It is important to know whether the cancer is SDH-deficient in order to plan treatment.

Stages of GIST

Key Points

  • After GIST has been diagnosed, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.

After GIST 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 GIST has spread to nearby areas or other parts of the body is called staging. GIST may spread to the lymph nodes, liver, and peritoneum. There is no standard staging system for childhood GIST. The results of tests and procedures done to diagnose GIST are used to help make decisions about treatment.

Sometimes childhood GIST recurs (comes back) after treatment.

There are three ways that cancer spreads in the body.

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

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

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.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if GIST spreads to the liver, the cancer cells in the liver are actually GIST cells. The disease is metastatic GIST, not liver cancer.

Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.

Treatment Option Overview

Key Points

  • There are different types of treatment for children with GIST.
  • Children with GIST should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
  • Two types of standard treatment are used:
    • Surgery
    • Targeted therapy
  • New types of treatment are being tested in clinical trials.
    • Chemotherapy
  • Treatment for childhood GIST may cause side effects.
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.

There are different types of treatment for children with GIST.

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 GIST 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:

Two types of standard treatment are used:

Surgery

Surgery to remove the cancer is a treatment for SDH-deficient GIST.

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.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Chemotherapy

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).

Treatment for childhood GIST may cause side effects.

To learn more about side effects that begin during treatment for cancer, visit Side Effects.

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:

  • Physical problems.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer) or other conditions.

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.

As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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).

Treatment of GIST

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

Treatment for children who have newly diagnosed tumors with changes in the KIT or PDGFR gene includes the following:

Treatment for children whose newly diagnosed tumors are SDH-deficient may include the following:

  • Surgery to remove the tumor. More surgery may be needed if intestinal obstruction or bleeding occurs.
  • Targeted therapy with a tyrosine kinase inhibitor (sunitinib).

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 GIST

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

Treatment of recurrent GIST in children may include the following:

To Learn More About GIST

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 stromal 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).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Gastrointestinal Stromal Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/soft-tissue-sarcoma/patient/child-gist-treatment-pdq. Accessed <MM/DD/YYYY>.

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

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More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Gastrointestinal Neuroendocrine Tumors—Patient Version

Gastrointestinal Neuroendocrine Tumors—Patient Version

Overview

Gastrointestinal (GI) neuroendocrine tumors are slow-growing tumors that form in the GI tract, mainly in the rectum, small intestine, or appendix. Explore the links on this page to learn more about GI neuroendocrine tumor treatment and clinical trials.

 

Causes & Prevention

NCI does not have PDQ evidence-based information about prevention of gastrointestinal neuroendocrine tumors.

Screening

NCI does not have PDQ evidence-based information about screening for gastrointestinal neuroendocrine tumors.

Coping with Cancer

The information in this section is meant to help you cope with the many issues and concerns that occur when you have cancer.

Emotions and Cancer Adjusting to Cancer Support for Caregivers Survivorship Advanced Cancer Managing Cancer Care

Colorectal Cancer—Patient Version

Colorectal Cancer—Patient Version

Overview

Colorectal cancer often begins as a growth called a polyp inside the colon or rectum. Finding and removing polyps can prevent colorectal cancer. Explore the links on this page to learn more about colorectal cancer prevention, screening, treatment, statistics, research, clinical trials, and more.

Causes & Prevention

Coping with Cancer

The information in this section is meant to help you cope with the many issues and concerns that occur when you have cancer.

Emotions and Cancer Adjusting to Cancer Support for Caregivers Survivorship Advanced Cancer Managing Cancer Care

Childhood Gastrointestinal Neuroendocrine Tumors Treatment (PDQ®)–Patient Version

Childhood Gastrointestinal Neuroendocrine Tumors Treatment (PDQ®)–Patient Version

General Information About Childhood Gastrointestinal Neuroendocrine Tumors

Key Points

  • A gastrointestinal neuroendocrine tumor forms in neuroendocrine cells in the lining of the digestive tract, appendix, and other organs in the abdomen.
  • Signs and symptoms of gastrointestinal neuroendocrine tumors depend on where the tumor forms.
  • Tests that examine the digestive tract, liver, and pancreas are used to diagnose gastrointestinal neuroendocrine tumors.
  • Certain factors affect prognosis (chance of recovery).

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

Gastrointestinal neuroendocrine tumors (also called 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 neuroendocrine 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.

EnlargeDrawing of the gastrointestinal tract showing the liver, stomach, pancreas, small intestine, colon, and appendix.
Gastrointestinal neuroendocrine tumors form in the lining of the gastrointestinal tract and other organs in the abdomen. Most gastrointestinal neuroendocrine tumors in children form in the appendix, but they can also form in the stomach, intestines, pancreas, and liver.

Signs and symptoms of gastrointestinal neuroendocrine 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 neuroendocrine tumors or by other conditions.

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

Gastrointestinal neuroendocrine tumors that are not in the appendix may release hormones and other substances. Carcinoid syndrome occurs when a neuroendocrine 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:

  • Redness and a warm feeling in the face, neck, and upper chest.
  • A fast heartbeat.
  • Trouble breathing.
  • Sudden drop in blood pressure (restlessness, confusion, weakness, dizziness, and pale, cool, and clammy skin).
  • Diarrhea.

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

The following tests and procedures may be used:

  • Physical exam and health 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 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.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet and radio waves to make a series of detailed pictures of areas inside the body. The pictures are made by a computer. This procedure is also called nuclear magnetic resonance imaging (NMRI).
    EnlargeMagnetic resonance imaging (MRI) scan; drawing shows a child lying on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body.
    Magnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.
  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
    EnlargePositron emission tomography (PET) scan; drawing shows a child lying on table that slides through the PET scanner.
    Positron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child’s vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
    EnlargeComputed tomography (CT) scan; drawing shows a child lying on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
    Computed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
  • Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
    EnlargeAbdominal ultrasound; drawing shows a child lying on an exam table during an abdominal ultrasound procedure. A technician is shown pressing a transducer (a device that makes sound waves that bounce off tissues inside the body) against the skin of the abdomen. A computer screen shows a sonogram (picture).
    Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).
  • Twenty-four-hour urine test: A test in which urine is collected for 24 hours 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 makes it. The urine sample is checked to see if it contains 5-HIAA (a breakdown product of the hormone serotonin which may be made by neuroendocrine tumors). This test is used to help diagnose carcinoid syndrome.
  • Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find tumors. A very small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.

Certain factors affect prognosis (chance of recovery).

Prognosis depends on the following:

  • Where the tumor first formed in the body.
  • The size of the tumor.
  • Whether the tumor has spread to other parts of the body.
  • Whether the tumor is newly diagnosed or has recurred (come back).

The prognosis for neuroendocrine tumors in the appendix in children is usually excellent after surgery to remove the tumor. Gastrointestinal neuroendocrine 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 Neuroendocrine Tumors

Key Points

  • After a gastrointestinal neuroendocrine 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.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.

After a gastrointestinal neuroendocrine 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 cancer has spread to nearby areas or other parts of the body is called staging. There is no standard staging system for childhood gastrointestinal neuroendocrine tumors.

Neuroendocrine tumors of the appendix are not known to spread, but other gastrointestinal neuroendocrine tumors may spread. The results of tests and procedures done to diagnose gastrointestinal neuroendocrine tumors are used to help make decisions about treatment.

Sometimes childhood gastrointestinal neuroendocrine 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:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

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.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

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

Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.

Treatment Option Overview

Key Points

  • There are different types of treatment for children with gastrointestinal neuroendocrine tumors.
  • Children with gastrointestinal neuroendocrine tumors not in the appendix should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
  • Five types of standard treatment are used:
    • Surgery
    • Embolization
    • Hormone therapy
    • Peptide receptor radionuclide therapy
    • Targeted therapy
  • New types of treatment are being tested in clinical trials.
  • Treatment of gastrointestinal neuroendocrine tumors may cause side effects.
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.

There are different types of treatment for children with gastrointestinal neuroendocrine 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 neuroendocrine 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 neuroendocrine 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 neuroendocrine tumors that have spread or cannot be removed by surgery. This treatment stops extra hormones from being made by the neuroendocrine 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 identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.

  • Tyrosine kinase inhibitors: These targeted therapy drugs block signals needed for tumors to grow. Sunitinib is used to treat gastrointestinal neuroendocrine tumors that are not in the appendix.
  • mTOR inhibitors: A type of targeted therapy that stops the protein that helps cells divide and survive. Everolimus is used to treat gastrointestinal neuroendocrine tumors that are not in the appendix.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment of gastrointestinal neuroendocrine tumors may cause side effects.

To learn more about side effects that begin during treatment for cancer, visit Side Effects.

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:

  • Physical problems.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer) or other conditions.

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.

As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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).

Treatment of Gastrointestinal Neuroendocrine Tumors

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

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

Treatment of newly diagnosed neuroendocrine 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:

Sometimes childhood gastrointestinal neuroendocrine tumors can recur (come back) after treatment. Your child’s doctor will work with you to plan treatment if your child is diagnosed with a recurrent gastrointestinal neuroendocrine tumor.

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 Neuroendocrine Tumors

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 neuroendocrine 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).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

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

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The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

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Childhood Colorectal Cancer Treatment (PDQ®)–Patient Version

Childhood Colorectal Cancer Treatment (PDQ®)–Patient Version

General Information About Childhood Colorectal Cancer

Key Points

  • Colorectal cancer is a disease in which malignant (cancer) cells form in the tissues of the colon or the rectum.
  • Having certain inherited syndromes increases the risk of colorectal cancer.
  • Signs and symptoms of colorectal cancer include pain in the abdomen, constipation, and diarrhea.
  • Tests that examine the colon and rectum are used to diagnose colorectal cancer.
  • Certain factors affect prognosis (chance of recovery).

Colorectal cancer is a disease in which malignant (cancer) cells form in the tissues of the colon or the rectum.

The colon is part of the body’s digestive system. The digestive system removes and processes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) from foods and helps pass waste material out of the body. The digestive system is made up of the mouth, throat, esophagus, stomach, and the small and large intestines. The colon (large bowel) is the main part of the large intestine and is about 5 feet long in an adult. Together, the rectum and anal canal make up the last part of the large intestine and are 6 to 8 inches long. The anal canal ends at the anus (the opening of the large intestine to the outside of the body).

Having certain inherited syndromes increases the risk of colorectal cancer.

Anything that increases your chance 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 child’s doctor if you think your child may be at risk.

Childhood colorectal cancer may be part of an inherited syndrome. Some colorectal cancers in young people are linked to a gene mutation that causes polyps (growths in the mucous membrane that lines the colon) to form that may turn into cancer later. The risk of colorectal cancer is increased by having inherited conditions, such as the following:

Polyps that form in the colon of children who do not have an inherited syndrome are not linked to an increased risk of cancer.

Signs and symptoms of colorectal cancer include pain in the abdomen, constipation, and diarrhea.

Signs and symptoms of childhood colorectal cancer usually depend on where the tumor forms. These and other signs and symptoms may be caused by colorectal cancer or by other conditions.

Check with your child’s doctor if your child has any of the following:

  • Tumors of the rectum or lower colon may cause pain in the abdomen, constipation, or diarrhea.
  • Tumors in the part of the colon on the left side of the body may cause:
    • A lump in the abdomen.
    • Weight loss for no known reason.
    • Nausea and vomiting.
    • Loss of appetite.
    • Blood in the stool.
    • Anemia (tiredness, dizziness, fast or irregular heartbeat, shortness of breath, pale skin).
  • Tumors in the part of the colon on the right side of the body may cause:
    • Pain in the abdomen.
    • Blood in the stool.
    • Constipation or diarrhea.
    • Nausea and vomiting.
    • Weight loss for no known reason.

Tests that examine the colon and rectum are used to diagnose colorectal cancer.

The following tests and procedures may be used:

  • Physical exam and health 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.
  • Colonoscopy: A procedure to look inside the rectum and colon for polyps, abnormal areas, or cancer. A colonoscope is inserted through the rectum into the colon. A colonoscope is a thin, tube-like instrument with a light and a lens for viewing. It also has a tool to remove polyps or tissue samples, which are checked under a microscope for signs of cancer.
  • Barium enema: A series of x-rays of the lower gastrointestinal tract. A liquid that contains barium (a silver-white metallic compound) is put into the rectum. The barium coats the lower gastrointestinal tract and x-rays are taken. This procedure is also called a lower GI series.
  • Fecal occult blood test: A test to check stool (solid waste) for blood that can only be seen with a microscope. Small samples of stool are placed on special cards and returned to the doctor or laboratory for testing.
  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
  • Carcinoembryonic antigen (CEA) assay: A test that measures the level of CEA in the blood. CEA is released into the bloodstream from both cancer cells and normal cells. When found in higher than normal amounts, it can be a sign of colorectal cancer or other conditions.

Certain factors affect prognosis (chance of recovery).

Prognosis depends on the following:

Stages of Childhood Colorectal Cancer

Key Points

  • After colorectal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the colon or rectum or to other parts of the body.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.
  • Sometimes childhood colorectal cancer recurs (comes back) after treatment.

After colorectal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the colon or rectum or to other parts of the body.

To plan treatment, it is important to know whether cancer cells have spread within the colon or rectum or to other parts of the body. The process used to find out if cancer has spread is called staging. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section.)

In children, the cancer has often spread to the lymph nodes, outside the colon or rectum, or to other organs in the abdomen at diagnosis. The following tests and procedures may be used to find out if cancer has spread:

  • Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the chest, abdomen, and pelvis. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner.
    EnlargeBone scan; drawing shows a child lying on a table that slides under the scanner, a technician operating the scanner, and a computer monitor that will show images made during the scan.
    Bone scan. A small amount of radioactive material is injected into the child’s vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen.

There are three ways that cancer spreads in the body.

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

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

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.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if colorectal cancer spreads to the bone, the cancer cells in the bone are actually colorectal cancer cells. The disease is metastatic colorectal cancer, not bone cancer.

Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.

Sometimes childhood colorectal cancer recurs (comes back) after treatment.

Childhood colorectal cancer may come back in the colon, rectum, or in other parts of the body.

Treatment Option Overview

Key Points

  • There are different types of treatment for children with colorectal cancer.
  • Children with colorectal cancer should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
  • Four types of standard treatment are used:
    • Surgery
    • Radiation therapy
    • Chemotherapy
    • Immunotherapy
  • New types of treatment are being tested in clinical trials.
  • Treatment for childhood colorectal cancer may cause side effects.
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.

There are different types of treatment for children with colorectal cancer.

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 colorectal cancer 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:

Four types of standard treatment are used:

Surgery

Surgery to remove the cancer is done if the cancer has not spread to other parts of the body at diagnosis.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.

Chemotherapy

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). Chemotherapy using more than one drug is called combination chemotherapy.

Immunotherapy

Immunotherapy 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 cancer treatment is a type of biologic therapy.

Immune checkpoint inhibitor therapy is a type of immunotherapy. Some types of immune cells, such as T cells, and some cancer cells have certain proteins, called checkpoint proteins, on their surface that keep immune responses in check. When cancer cells have large amounts of these proteins, they will not be attacked and killed by T cells. Immune checkpoint inhibitors block these proteins and the ability of T cells to kill cancer cells is increased.

Immunotherapy uses the body’s immune system to fight cancer. This animation explains one type of immunotherapy that uses immune checkpoint inhibitors to treat cancer.

There are two types of immune checkpoint inhibitor therapy:

  • PD-1 and PD-L1 inhibitor therapy: PD-1 is a protein on the surface of T cells that helps keep the body’s immune responses in check. PD-L1 is a protein found on some types of cancer cells. When PD-1 attaches to PD-L1, it stops the T cell from killing the cancer cell. PD-1 and PD-L1 inhibitors keep PD-1 and PD-L1 proteins from attaching to each other. This allows the T cells to kill cancer cells. Nivolumab is a type of PD-1 inhibitor that has been used to treat children 12 years and older with progressive colorectal cancer.
    EnlargeImmune checkpoint inhibitor; the panel on the left shows the binding of proteins PD-L1 (on the tumor cell) to PD-1 (on the T cell), which keeps T cells from killing tumor cells in the body. Also shown are a tumor cell antigen and T cell receptor. The panel on the right shows immune checkpoint inhibitors (anti-PD-L1 and anti-PD-1) blocking the binding of PD-L1 to PD-1, which allows the T cells to kill tumor cells.
    Immune checkpoint inhibitor. Checkpoint proteins, such as PD-L1 on tumor cells and PD-1 on T cells, help keep immune responses in check. The binding of PD-L1 to PD-1 keeps T cells from killing tumor cells in the body (left panel). Blocking the binding of PD-L1 to PD-1 with an immune checkpoint inhibitor (anti-PD-L1 or anti-PD-1) allows the T cells to kill tumor cells (right panel).
  • CTLA-4 inhibitor. CTLA-4 is a protein on the surface of T cells that helps keep the body’s immune responses in check. When CTLA-4 attaches to another protein called B7 on a cancer cell, it stops the T cell from killing the cancer cell. CTLA-4 inhibitors attach to CTLA-4 and allow the T cells to kill cancer cells. Ipilimumab is used to treat children 12 years and older with progressive colorectal cancer.
    EnlargeImmune checkpoint inhibitor; the panel on the left shows the binding of the T-cell receptor (TCR) to antigen and MHC proteins on the antigen-presenting cell (APC) and the binding of CD28 on the T cell to B7-1/B7-2 on the APC. It also shows the binding of B7-1/B7-2 to CTLA-4 on the T cell, which keeps the T cells in the inactive state. The panel on the right shows immune checkpoint inhibitor (anti-CTLA antibody) blocking the binding of B7-1/B7-2 to CTLA-4, which allows the T cells to be active and to kill tumor cells.
    Immune checkpoint inhibitor. Checkpoint proteins, such as B7-1/B7-2 on antigen-presenting cells (APC) and CTLA-4 on T cells, help keep the body’s immune responses in check. When the T-cell receptor (TCR) binds to antigen and major histocompatibility complex (MHC) proteins on the APC and CD28 binds to B7-1/B7-2 on the APC, the T cell can be activated. However, the binding of B7-1/B7-2 to CTLA-4 keeps the T cells in the inactive state so they are not able to kill tumor cells in the body (left panel). Blocking the binding of B7-1/B7-2 to CTLA-4 with an immune checkpoint inhibitor (anti-CTLA-4 antibody) allows the T cells to be active and to kill tumor cells (right panel).

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment for childhood colorectal cancer may cause side effects.

To learn more about side effects that begin during treatment for cancer, visit Side Effects.

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 the following:

  • Physical problems.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer) or other conditions.

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.

As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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).

Treatment of Childhood Colorectal Cancer

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

Treatment of newly diagnosed colorectal cancer in children may include the following:

Children with certain familial colorectal cancer syndromes may be treated with the following:

  • Surgery to remove the colon before cancer forms.
  • Medicine to decrease the number of polyps in the colon.

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 or Progressive Childhood Colorectal Cancer

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

Treatment of recurrent or progressive colorectal cancer 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 Childhood Colorectal Cancer

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 colorectal cancer. 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).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Colorectal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/colorectal/patient/child-colorectal-treatment-pdq. Accessed <MM/DD/YYYY>.

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Pancreatic Cancer Treatment (PDQ®)–Patient Version

Pancreatic Cancer Treatment (PDQ®)–Patient Version

General Information About Pancreatic Cancer

Key Points

  • Pancreatic cancer is a type of cancer that forms in the tissues of the pancreas.
  • Smoking and health history can affect the risk of pancreatic cancer.
  • Signs and symptoms of pancreatic cancer include jaundice, pain, and weight loss.
  • Pancreatic cancer is difficult to diagnose early.
  • Tests that examine the pancreas are used to diagnose and stage pancreatic cancer.
  • Some people decide to get a second opinion.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

Pancreatic cancer is a type of cancer that forms in the tissues 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 between the stomach and the spine.

EnlargeAnatomy of the pancreas; drawing shows the pancreas, stomach, spleen, liver, bile ducts, gallbladder, small intestine, and colon. An inset shows the head, body, and tail of the pancreas. The bile duct and pancreatic duct are also shown.
Anatomy of the pancreas. The pancreas has three areas: the head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs.

The pancreas has two main jobs in the body:

  • to make juices that help break down food into substances the body can use
  • to make hormones, such as insulin and glucagon, that help control blood sugar levels and help the body use and store the energy it gets from food

Pancreatic cancer can occur in exocrine pancreas cells, which produce digestive juices, or the endocrine pancreas cells, which produce hormones. About 95% of pancreatic cancers begin in exocrine cells.

This summary is about exocrine pancreatic cancer. For information about endocrine pancreatic cancer, visit Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment.

Smoking and health history can affect the risk of pancreatic cancer.

Pancreatic cancer is caused by certain changes to the way pancreatic cells function, especially how they grow and divide into new cells. A risk factor is anything that increases the chance of getting a disease. Some risk factors for pancreatic cancer, like smoking, can be changed. However, risk factors also include things people cannot change, like their genetics and their family history. Learning about risk factors for pancreatic cancer can help you make changes that might lower your risk of getting it.

There are many risk factors for pancreatic cancer, but many do not directly cause cancer. Instead, they increase the chance of DNA damage in cells that may lead to pancreatic cancer. Learn more about how cancer develops at What Is Cancer?

Having one or more of these risk factors does not mean that you will get pancreatic cancer. Many people with risk factors never develop pancreatic cancer, while others with no known risk factors do.

Risk factors for pancreatic cancer include:

Talk with your doctor if you think you might be at risk.

Signs and symptoms of pancreatic cancer include jaundice, pain, and weight loss.

Early on, pancreatic cancer may not cause any signs or symptoms, making it hard to detect. As the cancer grows, symptoms may include:

  • jaundice (yellowing of the skin and whites of the eyes)
  • light-colored stools
  • dark urine
  • pain in the upper or middle abdomen and back
  • weight loss for no known reason
  • loss of appetite
  • fatigue

These symptoms may be caused by many conditions other than pancreatic cancer. It’s important to check with your doctor if you have any of these symptoms to find out the cause and begin treatment, if needed.

Pancreatic cancer is difficult to diagnose early.

Pancreatic cancer is difficult to detect and diagnose for the following reasons:

  • There aren’t any noticeable signs or symptoms in the early stages of pancreatic cancer.
  • The signs and symptoms of pancreatic cancer, when present, are like the signs and symptoms of many other illnesses.
  • The pancreas is hidden behind other organs such as the stomach, small intestine, liver, gallbladder, spleen, and bile ducts.

Tests that examine the pancreas are used to diagnose and stage pancreatic cancer.

Pancreatic cancer is usually diagnosed with tests and procedures that make pictures of the pancreas and the area around it. The process used to find out if cancer cells have spread within and around the pancreas is called staging. Tests and procedures to detect, diagnose, and stage pancreatic cancer are usually done at the same time. To plan treatment, it is important to know the stage of the disease and whether the pancreatic cancer can be removed by surgery.

In addition to asking about your personal and family health history and doing a physical exam, your doctor may perform the following tests and procedures:

  • Blood chemistry study is a laboratory test in which a blood sample is checked to measure the amounts of certain substances, such as bilirubin, 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.
  • Tumor marker test checks a sample of blood, urine, or tissue to measure the amounts of certain substances, such as CA 19-9 and carcinoembryonic antigen (CEA), made by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the body. These are called tumor markers.
  • MRI (magnetic resonance imaging) uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • CT scan (CAT scan) uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. A spiral or helical CT scan makes a series of very detailed pictures of areas inside the body using an x-ray machine that scans the body in a spiral path.
  • PET scan (positron emission tomography scan) uses a small amount of radioactive sugar (also called glucose) that is injected into a vein. Then a scanner rotates around the body to make detailed, computerized pictures of areas inside the body where the glucose is taken up. Because cancer cells often take up more glucose than normal cells, the pictures can be used to find cancer cells in the body. A PET scan and CT scan may be done at the same time. This is called a PET-CT.
  • Abdominal ultrasound makes pictures of the inside of the abdomen. The ultrasound transducer is pressed against the skin of the abdomen and directs high-energy sound waves (ultrasound) into the abdomen. The sound waves bounce off the internal tissues and organs and make echoes. The transducer receives the echoes and sends them to a computer, which uses the echoes to make pictures called sonograms.
  • Endoscopic ultrasound (EUS) is a procedure that uses an endoscope inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography.
  • Endoscopic retrograde cholangiopancreatography (ERCP) is a procedure to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes pancreatic cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope (a thin, lighted tube) is passed through the mouth, esophagus, and stomach into the first part of the small intestine. A catheter (a smaller tube) is then inserted through the endoscope into the pancreatic ducts. A dye is injected through the catheter into the ducts and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube may be inserted into the duct to unblock it. This tube (or stent) may be left in place to keep the duct open. Tissue samples may also be taken.
  • Percutaneous transhepatic cholangiography (PTC) is a procedure to x-ray the liver and bile ducts. A thin needle is inserted through the skin below the ribs and into the liver. Dye is injected into the liver or bile ducts and an x-ray is taken. If a blockage is found, a thin, flexible tube called a stent is sometimes left in the liver to drain bile into the small intestine or a collection bag outside the body. This test is done only if ERCP cannot be done.
  • Laparoscopy is a surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. The laparoscope may have an ultrasound probe at the end in order to bounce high-energy sound waves off internal organs, such as the pancreas. This is called laparoscopic ultrasound. Other instruments may be inserted through the same or other incisions to perform procedures such as taking tissue samples from the pancreas or a sample of fluid from the abdomen to check for cancer.
  • Biopsy is the removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. There are several ways to do a biopsy for pancreatic cancer. A fine needle or a core needle may be inserted into the pancreas during an x-ray or ultrasound to remove cells. Tissue may also be removed during a laparoscopy or surgery to remove the tumor.

Some people decide to get a second opinion.

You may want to get a second opinion to confirm your pancreatic cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. They may agree with the first doctor, suggest changes or another treatment approach, or provide more information about your cancer.

To learn more about choosing a doctor and getting a second opinion, visit Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor, hospital, or getting a second opinion. For questions you might want to ask at your appointments, visit Questions to Ask Your Doctor about Cancer.

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

The prognosis and treatment options depend on:

  • whether the tumor can be removed by surgery
  • the stage of the cancer (the size of the tumor and whether the cancer has spread outside the pancreas to nearby tissues or lymph nodes or to other places in the body)
  • the patient’s general health
  • whether the cancer has just been diagnosed or has recurred (come back)

Pancreatic cancer can be controlled only if it is found before it has spread, when it can be completely removed by surgery. If the cancer has spread, palliative treatment can improve the patient’s quality of life by controlling the symptoms and complications of this disease.

Stages of Pancreatic Cancer

Key Points

  • Cancer stage describes the extent of cancer in the body.
  • The following stages are used for pancreatic cancer:
    • Stage 0 (carcinoma in situ)
    • Stage I (also called stage 1) pancreatic cancer
    • Stage II (also called stage 2) pancreatic cancer
    • Stage III (also called stage 3) pancreatic cancer
    • Stage IV (also called stage 4) pancreatic cancer
  • The following groups are used to plan treatment:
    • Resectable pancreatic cancer
    • Borderline resectable pancreatic cancer
    • Locally advanced pancreatic cancer
    • Metastatic pancreatic cancer
    • Recurrent pancreatic cancer

Cancer stage describes the extent of cancer in the body.

Cancer stage describes the extent of cancer in the body, such as the size of the tumor, whether it has spread, and how far it has spread from where it first formed. It is important to know the stage of the pancreatic cancer to plan the best treatment.

There are several staging systems for cancer that describe the extent of the cancer. Pancreatic cancer staging usually uses the TNM staging system. Your cancer may be described by this staging system in your pathology report. Based on the TNM results, a stage (I, II, III, or IV, also written as 1, 2, 3, or 4) is assigned to your cancer. When talking to you about your cancer, your doctor may describe it as one of these stages.

To learn how doctors stage pancreatic cancer, visit tests to diagnose and stage pancreatic cancer. Learn more about Cancer Staging.

The information in this section is about staging for exocrine pancreatic cancer, the most common type of pancreatic cancer.

The following stages are used for pancreatic cancer:

Stage 0 (carcinoma in situ)

EnlargeStage 0 pancreatic cancer; drawing shows abnormal cells in the pancreas.
Stage 0 pancreatic cancer. Abnormal cells are found in the lining of the pancreas. These abnormal cells may become cancer and spread into nearby normal tissue.

In stage 0, abnormal cells are found in the lining of the pancreas. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.

Stage I (also called stage 1) pancreatic cancer

EnlargeStage I pancreatic cancer; drawing on the left shows stage IA pancreatic cancer. The cancer is in the pancreas and the tumor is 2 centimeters or smaller. An inset shows 2 centimeters is about the size of a peanut. The drawing on the right shows stage IB pancreatic cancer. The cancer is in the pancreas and the tumor is larger than 2 centimeters but not larger than 4 centimeters. An inset shows 2 centimeters is about the size of a peanut and 4 centimeters is about the size of a walnut.
Stage I pancreatic cancer. Cancer is found in the pancreas only. In stage IA, the tumor is 2 centimeters or smaller. In stage IB, the tumor is larger than 2 centimeters but not larger than 4 centimeters.

In stage I, cancer has formed and is found in the pancreas only. Stage I is divided into stages IA and IB, depending on the size of the tumor.

  • Stage IA: The tumor is 2 centimeters or smaller.
  • Stage IB: The tumor is larger than 2 centimeters but not larger than 4 centimeters.

Stage II (also called stage 2) pancreatic cancer

Stage II is divided into stages IIA and IIB, depending on the size of the tumor and where the cancer has spread.

  • Stage IIA: The tumor is larger than 4 centimeters.
    EnlargeStage IIA pancreatic cancer; drawing shows cancer in the pancreas and the tumor is larger than 4 centimeters. An inset shows 4 centimeters is about the size of a walnut.
    Stage IIA pancreatic cancer. The tumor is larger than 4 centimeters.
  • Stage IIB: The tumor is any size, and cancer has spread to 1 to 3 nearby lymph nodes.
    EnlargeStage IIB pancreatic cancer; drawing shows cancer in the pancreas and in 1 to 3 nearby lymph nodes.
    Stage IIB pancreatic cancer. The tumor is any size and cancer has spread to 1 to 3 nearby lymph nodes.

Stage III (also called stage 3) pancreatic cancer

EnlargeStage III pancreatic cancer; drawing shows cancer in the pancreas and in (a) 4 or more nearby lymph nodes and (b) the common hepatic artery. Also shown are the portal vein, celiac axis (trunk), and superior mesenteric artery.
Stage III pancreatic cancer. The tumor is any size and cancer has spread to (a) 4 or more nearby lymph nodes; or (b) the major blood vessels near the pancreas. These include the portal vein, common hepatic artery, celiac axis (trunk), and superior mesenteric artery.

In stage III, the tumor is any size, and cancer has spread to:

Stage IV (also called stage 4) pancreatic cancer

EnlargeStage IV pancreatic cancer; drawing shows other parts of the body where pancreatic cancer may spread, including the lung, liver, and peritoneal cavity. An inset shows cancer cells spreading from the pancreas, through the blood and lymph system, to another part of the body where metastatic cancer has formed.
Stage IV pancreatic cancer. The tumor is any size and cancer has spread to other parts of the body, such as the lung, liver, or peritoneal cavity (the body cavity that contains most of the organs in the abdomen).

In stage IV, the tumor is any size, and cancer has spread to other parts of the body, such as the liver, lung, or peritoneal cavity (the body cavity that contains most of the organs in the abdomen).

Stage IV pancreatic cancer is also called metastatic pancreatic cancer. Metastatic cancer happens when cancer cells travel through the lymphatic system or blood and form tumors in other parts of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if pancreatic cancer spreads to the liver, the cancer cells in the liver are actually pancreatic cancer cells. The disease is called metastatic pancreatic cancer, not liver cancer. Learn more in Metastatic Cancer: When Cancer Spreads.

The following groups are used to plan treatment:

Resectable pancreatic cancer

Resectable pancreatic cancer can be removed by surgery because it has not grown into major blood vessels near the tumor.

Borderline resectable pancreatic cancer

Borderline resectable pancreatic cancer has grown into a major blood vessel or nearby tissue or organs. It may be possible to remove the tumor, but there is a high risk that all of the cancer cells will not be removed with surgery.

Locally advanced pancreatic cancer

Locally advanced pancreatic cancer has grown into or close to nearby lymph nodes or blood vessels, so surgery cannot completely remove the cancer.

Metastatic pancreatic cancer

Metastatic pancreatic cancer has spread to other organs, so surgery cannot completely remove the cancer.

Recurrent pancreatic cancer

Recurrent pancreatic cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the pancreas or in other parts of the body. Tests will be done to help determine where the cancer has returned in your body. The type of treatment for recurrent pancreatic cancer will depend on where it has come back.

Learn more in Recurrent Cancer: When Cancer Comes Back.

Treatment Option Overview

Key Points

  • There are different types of treatment for patients with pancreatic cancer.
  • The following types of treatment are used:
    • Surgery
    • Radiation therapy
    • Chemotherapy
    • Chemoradiation therapy
    • Targeted therapy
  • There are treatments for pain caused by pancreatic cancer.
  • Patients with pancreatic cancer have special nutritional needs.
  • New types of treatment are being tested in clinical trials.
  • Treatment for pancreatic cancer may cause side effects.
  • Follow-up care may be needed.

There are different types of treatment for patients with pancreatic cancer.

Different types of treatments are available for people with pancreatic cancer. You and your cancer care team will work together to decide your treatment plan, which may include more than one type of treatment. Many factors will be considered, such as the stage of the cancer, your overall health, and your preferences. Your plan will include information about your cancer, the goals of treatment, your treatment options and the possible side effects, and the expected length of treatment.

Talking with your cancer care team before treatment begins about what to expect will be helpful. You’ll want to learn what you need to do before treatment begins, how you’ll feel while going through it, and what kind of help you will need. To learn more, visit Questions to Ask Your Doctor about Treatment.

The following types of treatment are used:

Surgery

One of the following types of surgery may be used to take out the tumor:

  • Whipple procedure is surgery to remove the head of the pancreas, the gallbladder, part of the stomach, part of the small intestine, and the bile duct. Enough of the pancreas is left to produce digestive juices and insulin.
  • Total pancreatectomy is surgery to remove the whole pancreas, part of the stomach, part of the small intestine, the common bile duct, the gallbladder, the spleen, and nearby lymph nodes.
  • Distal pancreatectomy is surgery to remove the body and the tail of the pancreas. The spleen may also be removed if cancer has spread to the spleen.

If the cancer has spread and cannot be removed, the following types of palliative surgery may be done to relieve symptoms and improve quality of life:

  • Biliary bypass: If cancer is blocking the bile duct and bile is building up in the gallbladder, a biliary bypass may be done. During this operation, the doctor will cut the gallbladder or bile duct in the area before the blockage and sew it to the small intestine to create a new pathway around the blocked area.
  • Endoscopic stent placement: If the tumor is blocking the bile duct, surgery may be done to put in a stent (a thin tube) to drain bile that has built up in the area. The doctor may place the stent through a catheter that drains the bile into a bag on the outside of the body, or the stent may go around the blocked area and drain the bile into the small intestine.
  • Gastric bypass: If the tumor is blocking the flow of food from the stomach, the stomach may be sewn directly to the small intestine so the patient can continue to eat normally.

Radiation therapy

Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Pancreatic cancer is sometimes treated with external beam radiation therapy. This type of radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.

Learn more about External Beam Radiation Therapy for Cancer and Radiation Therapy Side Effects.

Chemotherapy

Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing.

Chemotherapy for pancreatic cancer is usually systemic, meaning it is injected into a vein or given by mouth. When given this way, the drugs enter the bloodstream to reach cancer cells throughout the body.

Chemotherapy drugs used to treat pancreatic cancer may include:

Combinations of these drugs may be used. Other chemotherapy drugs not listed here may also be used.

Chemotherapy may also be combined with other kinds of treatment. For example, it may be combined with radiation therapy or targeted therapy.

Learn more about how chemotherapy works, how it is given, common side effects, and more at Chemotherapy to Treat Cancer and Chemotherapy and You: Support for People With Cancer.

Chemoradiation therapy

Chemoradiation therapy combines chemotherapy and radiation therapy to increase the effects of both.

Targeted therapy

Targeted therapy uses drugs or other substances to identify and attack specific cancer cells. Erlotinib is a targeted therapy drug used to treat pancreatic cancer.

Learn more about Targeted Therapy to Treat Cancer. 

There are treatments for pain caused by pancreatic cancer.

Pain can occur when the tumor presses on nerves or other organs near the pancreas. When pain medicine is not enough, there are treatments that act on nerves in the abdomen to relieve the pain. The doctor may inject medicine into the area around affected nerves or may cut the nerves to block the feeling of pain. Radiation therapy with or without chemotherapy can also help relieve pain by shrinking the tumor. Learn more about Cancer Pain.

Patients with pancreatic cancer have special nutritional needs.

Surgery to remove the pancreas may affect its ability to make pancreatic enzymes that help to digest food. As a result, patients may have problems digesting food and absorbing nutrients into the body. To prevent malnutrition, the doctor may prescribe medicines that replace these enzymes.

New types of treatment are being tested in clinical trials.

For some people, joining a clinical trial may be an option. There are different types of clinical trials for people with cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.

You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.

Treatment for pancreatic cancer may cause side effects.

For information about side effects caused by treatment for cancer, visit our Side Effects page.

Follow-up care may be needed.

As you go through treatment, you will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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 condition has changed or if the cancer has recurred (come back).

Treatment of Resectable or Borderline Resectable Pancreatic Cancer

Treatment of resectable or borderline resectable pancreatic cancer may include:

  • chemotherapy with or without radiation therapy, followed by surgery
  • surgery
  • surgery, followed by chemotherapy
  • surgery, followed by chemoradiation
  • a clinical trial of chemotherapy and/or radiation therapy before surgery
  • a clinical trial of chemoradiation, followed by surgery and then chemotherapy
  • a clinical trial of different ways of giving radiation therapy

Surgery to remove the tumor may include Whipple procedure, total pancreatectomy, or distal pancreatectomy.

Palliative therapy can be started at any stage of disease. Visit the Palliative Therapy section for information about treatments that may improve quality of life or relieve symptoms in people with pancreatic cancer.

Learn more about these treatments in the Treatment Option Overview.

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 Locally Advanced Pancreatic Cancer

Treatment of pancreatic cancer that is locally advanced may include:

Palliative therapy can be started at any stage of disease. Visit the Palliative Therapy section for information about treatments that may improve quality of life or relieve symptoms in patients with pancreatic cancer.

Learn more about these treatments in the Treatment Option Overview.

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 Metastatic or Recurrent Pancreatic Cancer

Treatment of pancreatic cancer that has metastasized or recurred may include:

Palliative therapy can be started at any stage of disease. Visit the Palliative Therapy section for information about treatments that may improve quality of life or relieve symptoms in patients with pancreatic cancer.

Learn more about these treatments in the Treatment Option Overview.

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.

Palliative Therapy

Palliative therapy can improve the patient’s quality of life by controlling the symptoms and complications of pancreatic cancer.

Palliative therapy for pancreatic cancer may include:

Learn more about these treatments in the Treatment Option Overview.

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 Pancreatic Cancer

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 adult pancreatic cancer. 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 Adult 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).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Pancreatic Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/pancreatic/patient/pancreatic-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389396]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment (PDQ®)–Patient Version

Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment (PDQ®)–Patient Version

General Information About Childhood Multiple Endocrine Neoplasia (MEN) Syndromes

Key Points

  • Multiple endocrine neoplasia (MEN) syndromes are inherited disorders that affect the endocrine system.
  • There are several types of MEN syndromes and each type may cause different conditions or cancers.
  • MEN1 syndrome usually causes tumors in the parathyroid gland, pituitary gland, or islet cells of the pancreas.
  • MEN2A syndrome may cause medullary thyroid cancer, pheochromocytoma, or parathyroid gland disease.
  • MEN2B syndrome causes several conditions.
  • Children with MEN2A syndrome, MEN2B syndrome, or FMTC may need genetic testing.
  • Tests used to diagnose and stage cancers related to MEN syndromes depend on the signs and symptoms and the patient’s family history.
  • If cancer, such as medullary thyroid cancer, has formed, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.

Multiple endocrine neoplasia (MEN) syndromes are inherited disorders that affect the endocrine system.

The endocrine system is made up of glands and cells that make hormones and release them into the blood. MEN syndromes may cause hyperplasia (the growth of too many normal cells) or tumors that may be benign (not cancer) or malignant (cancer).

There are several types of MEN syndromes and each type may cause different conditions or cancers.

The two main types of MEN syndromes are MEN1 and MEN2. MEN2 syndrome has two subgroups: MEN2A syndrome and MEN2B syndrome. MEN2A syndrome includes familial medullary thyroid cancer.

EnlargeParts of the body affected by multiple endocrine neoplasia (MEN) syndromes; the drawing on the left shows parts of the body affected by MEN1 syndrome, including the pituitary gland, parathyroid glands, and pancreas. An inset shows the back view of the thyroid gland and the four pea-sized parathyroid glands. The drawing on the right shows parts of the body affected by MEN2 syndrome, including the thyroid gland, parathyroid glands, and adrenal gland. An inset shows the adrenal medulla (inner part) of the adrenal gland.
Multiple endocrine neoplasia (MEN) syndromes are rare, inherited disorders that affect the endocrine glands or organs. There are several types of MEN syndromes, and each type may cause different conditions or cancers. The two main types of MEN syndromes are MEN1 and MEN2. MEN1 syndrome usually causes tumors in the pituitary gland, parathyroid gland, or pancreas. MEN2 syndrome usually causes tumors in the thyroid gland, parathyroid gland, or adrenal gland. The tumors may be benign (not cancer) or malignant (cancer).

MEN1 syndrome usually causes tumors in the parathyroid gland, pituitary gland, or islet cells of the pancreas.

A diagnosis of MEN1 syndrome is made when tumors are found in two of the following glands or organs: parathyroid gland, pituitary gland, or islet cells in the pancreas. These tumors may make extra hormones and cause certain signs or symptoms of disease. The signs and symptoms depend on the type of hormone made by the tumor. MEN1 syndrome is also called Wermer syndrome.

The prognosis (chance of recovery) is usually good.

The most common condition associated with MEN1 syndrome is hyperparathyroidism. Signs and symptoms of hyperparathyroidism (too much parathyroid hormone) include the following:

  • Having a kidney stone.
  • Feeling weak or very tired.
  • Bone pain.

Other conditions associated with MEN1 syndrome and their common signs and symptoms are:

Children with primary hyperparathyroidism, tumors associated with MEN1 syndrome, or a family history of hypercalcemia or MEN1 syndrome may have genetic testing to check for a mutation (change) in the MEN1 gene. Parents should receive genetic counseling (a discussion with a trained professional about the risk of genetic diseases) before genetic testing is done. Genetic counseling also includes a discussion of the risk of MEN1 syndrome for the child and other family members.

Children who are diagnosed with MEN1 syndrome are checked for signs of cancer starting at age 5 years and continuing for the rest of their life. Talk to your child’s doctor about the tests and procedures needed to check for signs of cancer and how often they should be done.

MEN2A syndrome may cause medullary thyroid cancer, pheochromocytoma, or parathyroid gland disease.

MEN2A syndrome is also called Sipple syndrome. A diagnosis of MEN2A syndrome may be made when the patient or the patient’s parents, brothers, sisters, or children have two or more of the following conditions:

  • Medullary thyroid cancer (a cancer that forms in parafollicular C cells in the thyroid). Signs and symptoms of medullary thyroid cancer may include the following:
    • A lump in the throat or neck.
    • Trouble breathing.
    • Trouble swallowing.
    • Hoarseness.
  • Pheochromocytoma (a tumor of the adrenal gland). Signs and symptoms of pheochromocytoma may include the following:
    • High blood pressure.
    • Pain in the abdomen or chest.
    • A strong, fast, or irregular heartbeat.
    • Headache.
    • Heavy sweating for no known reason.
    • Dizziness.
    • Feeling shaky.
    • Being irritable or nervous.
  • Parathyroid gland disease (a benign tumor of the parathyroid gland or increase in the size of the parathyroid gland). Signs and symptoms of parathyroid gland disease may include the following:
    • Hypercalcemia.
    • Pain in the abdomen, side, or back that doesn’t go away.
    • Pain in the bones.
    • A broken bone.
    • A lump in the neck.
    • Trouble speaking.
    • Trouble swallowing.
  • Familial medullary cancer of the thyroid is like MEN2A syndrome without pheochromocytoma or parathyroid tumors. A diagnosis of familial medullary cancer of the thyroid may be made when two or more family members have medullary thyroid cancer and no family members have parathyroid or adrenal gland problems. Signs and symptoms of medullary thyroid cancer may include the following:
    • A lump in the throat or neck.
    • Trouble breathing.
    • Trouble swallowing.
    • Hoarseness.

The prognosis is usually good.

A mutation in the RET gene is usually linked to medullary thyroid cancer in MEN2 syndrome. If a diagnosis of MEN2 syndrome is suspected for the child or a family member is diagnosed with MEN2 syndrome, the parents should receive genetic counseling before genetic testing is done for the child. Genetic counseling also includes a discussion of the risk of MEN2 syndrome for the child and other family members.

MEN2B syndrome causes several conditions.

Patients with MEN2B syndrome may have a slender body build with long, thin arms and legs. The lips may appear large and bumpy because of benign tumors in the mucous membranes. MEN2B syndrome may cause the following conditions:

The prognosis for MEN2B syndrome is not as good as the prognosis for MEN1 syndrome and MEN2A syndrome because medullary thyroid cancer is a more aggressive cancer.

Children with MEN2A syndrome, MEN2B syndrome, or FMTC may need genetic testing.

A mutation in the RET gene is usually linked to medullary thyroid cancer in MEN2 syndrome. If a diagnosis of MEN2 syndrome is suspected for the child or a family member is diagnosed with MEN2 syndrome, the parents should receive genetic counseling before genetic testing is done for the child. Genetic counseling also includes a discussion of the risk of MEN2 syndrome for the child and other family members.

Tests used to diagnose and stage cancers related to MEN syndromes depend on the signs and symptoms and the patient’s family history.

Tests are done to diagnose and stage cancer. After cancer is diagnosed, more tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body. This process is called staging.

The following tests and procedures may be used:

  • Physical exam and health 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 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.
  • Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain 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 makes it. The blood may also be checked for high levels of the hormone calcitonin or parathyroid hormone (PTH).
  • Genetic testing: A laboratory test in which cells or tissue are analyzed to look for changes in genes or chromosomes. These changes may be a sign that a person has or is at risk of having a specific disease or condition. A sample of blood is checked for the MEN1 gene to diagnose MEN1 syndrome and for the RET gene to diagnose MEN2 syndrome.
  • Twenty-four-hour urine test: A procedure used to diagnose neuroendocrine tumors, such as pheochromocytoma. Urine is collected for 24 hours to measure the amounts of catecholamines in the urine. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher than normal amounts may be a sign of pheochromocytoma.
  • Ultrasound: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
  • MRI: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • CT scan: A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • PET scan: A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
  • Thyroid scan: A small amount of a radioactive substance is swallowed or injected. The radioactive material collects in thyroid gland cells. A special camera linked to a computer detects the radiation given off and makes pictures that show how the thyroid looks and functions and whether the cancer has spread beyond the thyroid gland. If the amount of thyroid-stimulating hormone in the child’s blood is low, a scan to make images of the thyroid may be done before surgery.
  • Sestamibi scan: A type of radionuclide scan used to find an overactive parathyroid gland. A very small amount of a radioactive substance called technetium 99 is injected into a vein and travels through the bloodstream to the parathyroid gland. The radioactive substance will collect in the overactive gland and show up brightly on a special camera that detects radioactivity.
  • Venous sampling for an overactive parathyroid gland: A procedure in which a sample of blood is taken from veins near the parathyroid glands. The sample is checked to measure the amount of parathyroid hormone released into the blood by each gland. Venous sampling may be done if blood tests show there is an overactive parathyroid gland but imaging tests don’t show which one it is.
  • Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find tumors. A very small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show whether there are islet cell tumors in the pancreas. This procedure is also called octreotide scan and SRS.
  • MIBG scan: A procedure used to find neuroendocrine tumors, such as pheochromocytoma. A very small amount of a substance called radioactive MIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive MIBG and are detected by a scanner. Scans may be taken over 1-3 days. An iodine solution may be given before or during the test to keep the thyroid gland from absorbing too much of the MIBG.
  • Pentagastrin stimulation test: A test in which blood samples are checked to measure the amount of calcitonin in the blood. Calcium gluconate and pentagastrin are injected into the blood and then several blood samples are taken over the next 5 minutes. If the level of calcitonin in the blood increases, it may be a sign of medullary thyroid cancer.
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.

If cancer, such as medullary thyroid cancer, has formed, 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 cancer has spread to nearby areas or to other parts of the body is called staging. There is no standard system for staging childhood cancers linked to MEN syndromes. The results of the tests and procedures done to diagnose cancer are used to help make decisions about treatment.

Sometimes childhood MEN syndromes 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:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

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.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if medullary thyroid cancer spreads to the lung, the cancer cells in the lung are actually medullary thyroid cells. The disease is metastatic medullary thyroid cancer, not lung cancer.

Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.

Treatment Option Overview

Key Points

  • There are different types of treatment for children with MEN syndromes.
  • Children with MEN syndromes should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
  • The treatment will depend on the type of MEN syndrome the child has.
  • New types of treatment are being tested in clinical trials.
  • Treatment for childhood MEN syndrome may cause side effects.
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.

There are different types of treatment for children with MEN syndromes.

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 MEN syndromes 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:

The treatment will depend on the type of MEN syndrome the child has.

See the treatment section of this summary for information on the treatment of MEN1 syndrome, MEN2A syndrome, and MEN2B syndrome.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment for childhood MEN syndrome may cause side effects.

To learn more about side effects that begin during treatment for cancer, visit Side Effects.

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.

As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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).

Treatment of Childhood MEN1 Syndrome

Hyperparathyroidism is the most common sign of MEN1 syndrome. Children with MEN1 syndrome and primary hyperparathyroidism may have surgery to remove at least three parathyroid glands and the thymus. Treatment is also given for pancreatic islet cell and pituitary tumors, and other conditions linked to MEN1 syndrome as needed.

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 Childhood MEN2A Syndrome

Medullary thyroid cancer is linked to MEN2A syndrome. Children with MEN2A syndrome and certain changes in the RET gene usually have surgery to remove the thyroid before the child is 5 years old to diagnose cancer or to lessen the chance cancer will form or spread. Treatment is also given for pheochromocytoma and hyperparathyroidism as needed.

Children with MEN2A syndrome who have medullary thyroid cancer may be treated with targeted therapy. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.

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 Childhood MEN2B Syndrome

Medullary thyroid cancer, an aggressive form of thyroid cancer, is also linked to MEN2B syndrome. Infants with MEN2B syndrome and certain changes in the RET gene usually have surgery to remove the thyroid to lessen the chance cancer will form.

Children with MEN2B syndrome who have medullary thyroid cancer may be treated with targeted therapy. Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.

Treatment is also given for pheochromocytoma and other conditions as needed.

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 Childhood MEN Syndromes

Treatment of cancer linked to MEN syndrome that has recurred (come back) may include the following:

To Learn More About Childhood MEN Syndromes

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 multiple endocrine neoplasia (MEN) syndromes. 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).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/multiple-endocrine-neoplasia/patient-child-men-syndromes-treatment-pdq. Accessed <MM/DD/YYYY>.

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Pancreatic Cancer—Patient Version

Pancreatic Cancer—Patient Version

Overview

Pancreatic cancer can develop from two kinds of cells in the pancreas: exocrine cells and neuroendocrine cells, such as islet cells. The exocrine type is more common and is usually found at an advanced stage. Pancreatic neuroendocrine tumors (islet cell tumors) are less common but have a better prognosis. Explore the links on this page to learn more about  pancreatic cancer treatment, statistics, research, and clinical trials.

Causes & Prevention

NCI does not have PDQ evidence-based information about prevention of pancreatic cancer.

Screening

NCI does not have PDQ evidence-based information about screening for pancreatic cancer.

Coping with Cancer

The information in this section is meant to help you cope with the many issues and concerns that occur when you have cancer.

Emotions and Cancer Adjusting to Cancer Support for Caregivers Survivorship Advanced Cancer Managing Cancer Care

Childhood Pancreatic Cancer Treatment (PDQ®)–Patient Version

Childhood Pancreatic Cancer Treatment (PDQ®)–Patient Version

General Information About Childhood Pancreatic Cancer

Key Points

  • Pancreatic cancer is a disease in which malignant (cancer) cells form in the tissues of the pancreas.
  • There are four types of pancreatic cancer in children.
  • Signs and symptoms of pancreatic cancer include feeling tired and weight loss.
  • Tests that examine the pancreas are used to help diagnose pancreatic cancer.

Pancreatic cancer is a disease in which malignant (cancer) cells form in the tissues 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 between the stomach and the spine.

EnlargeAnatomy of the pancreas; drawing shows the pancreas, stomach, spleen, liver, bile ducts, gallbladder, small intestine, and colon. An inset shows the head, body, and tail of the pancreas and the bile duct and pancreatic duct.
Anatomy of the pancreas. The pancreas has three parts: the head, the body, and the tail. It is found in the abdomen near the stomach, intestines, and other organs.

There are two kinds of cells in the pancreas:

There are four types of pancreatic cancer in children.

The four types of pancreatic cancer in children include the following:

Signs and symptoms of pancreatic cancer include feeling tired and weight loss.

General signs and symptoms of pancreatic cancer may include the following:

  • Feeling tired.
  • Weight loss for no known reason.
  • Loss of appetite.
  • Stomach discomfort.
  • Lump in the abdomen.

In children, some pancreatic tumors do not secrete hormones and there are no signs and symptoms of disease. This makes it hard to diagnose pancreatic cancer early.

Pancreatic tumors that do secrete hormones may cause signs and symptoms. The signs and symptoms depend on the type of hormone being made.

If the tumor secretes insulin, signs and symptoms that may occur include the following:

  • Low blood sugar. This can cause blurred vision, headache, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry.
  • Changes in behavior.
  • Seizures.
  • Coma.

If the tumor secretes gastrin, signs and symptoms that may occur include the following:

  • Stomach ulcers that keep coming back.
  • Pain in the abdomen, which may spread to the back. The pain may come and go and it may go away after taking an antacid.
  • The flow of stomach contents back into the esophagus (gastroesophageal reflux).
  • Diarrhea.

Signs and symptoms caused by tumors that make other types of hormones, such as ACTH or ADH, may include the following:

  • Watery diarrhea.
  • Dehydration (feeling thirsty, making less urine, dry skin and mouth, headaches, dizziness, or feeling tired).
  • Low sodium (salt) level in the blood (confusion, sleepiness, muscle weakness, and seizures).
  • Weight loss or gain for no known reason.
  • Round face and thin arms and legs.
  • Feeling very tired and weak.
  • High blood pressure.
  • Purple or pink stretch marks on the skin.

Check with your child’s doctor if you see any of these problems in your child. Other conditions that are not pancreatic cancer may cause these same signs and symptoms.

Tests that examine the pancreas are used to help diagnose pancreatic cancer.

The following tests and procedures may be used:

  • Physical exam and health 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.
  • Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest and abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
    EnlargeComputed tomography (CT) scan; drawing shows a child lying on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
    Computed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the chest and abdomen. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
    EnlargePositron emission tomography (PET) scan; drawing shows a child lying on table that slides through the PET scanner.
    Positron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child’s vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
  • Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography.
  • Somatostatin receptor scintigraphy: A type of radionuclide scan used to find pancreatic tumors. A very small amount of radioactive octreotide (a hormone that attaches to carcinoid tumors) is injected into a vein and travels through the bloodstream. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is used to diagnose islet cell tumors.
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. Procedures used to obtain a tissue sample include the following:
    • Core-needle biopsy: A procedure to remove tissue using a wide needle.
    • Laparoscopy: A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as taking tissue samples.
    • Laparotomy: A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes tissue samples are taken.

Stages of Childhood Pancreatic Cancer

Key Points

  • If cancer has formed in the pancreas, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.

If cancer has formed in the pancreas, 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 cancer has spread from the pancreas to nearby areas or to other parts of the body is called staging. There is no standard system for staging childhood pancreatic cancer. The results of the tests and procedures done to diagnose cancer are used to help make decisions about treatment.

Sometimes childhood pancreatic cancer recurs (comes back) after treatment. It may come back in the pancreas or other parts of the body.

There are three ways that cancer spreads in the body.

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

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

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.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if pancreatic cancer spreads to the lung, the cancer cells in the lung are actually pancreas cancer cells. The disease is metastatic pancreatic cancer, not lung cancer.

Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.

Treatment Option Overview

Key Points

  • There are different types of treatment for children with pancreatic cancer.
  • Children with pancreatic cancer should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
  • Three types of standard treatment are used:
    • Surgery
    • Chemotherapy
    • Targeted therapy
  • New types of treatment are being tested in clinical trials.
  • Treatment for childhood pancreatic cancer may cause side effects.
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.

There are different types of treatment for children with pancreatic cancer.

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 pancreatic cancer 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:

Three types of standard treatment are used:

Surgery

Surgery to remove the tumor is used to treat most types of pancreatic cancer. For cancer in the head of the pancreas, a Whipple procedure may be done.

Chemotherapy

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).

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific 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 for childhood pancreatic cancer may cause side effects.

To learn more about side effects that begin during treatment for cancer, visit Side Effects.

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:

  • Physical problems.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer) or other conditions.

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.

As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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).

Treatment of Childhood Pancreatic Cancer

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

Treatment of newly diagnosed solid pseudopapillary tumor of the pancreas in children may include the following:

  • Surgery to remove the tumor. A Whipple procedure may be done for tumors in the head of the pancreas.
  • Chemotherapy for tumors that cannot be removed by surgery or have spread to other parts of the body.

Treatment of newly diagnosed pancreatoblastoma in children may include the following:

  • Surgery to remove the tumor. A Whipple procedure may be done for tumors in the head of the pancreas.
  • Chemotherapy may be given to shrink the tumor before surgery. More chemotherapy may be given after surgery for large tumors, tumors that could not initially be removed by surgery, and tumors that have spread to other parts of the body.
  • Chemotherapy may be given if the tumor does not respond to treatment or comes back.

Treatment of newly diagnosed islet cell tumors in children may include drugs to treat symptoms caused by hormones and the following:

There are few reported cases of pancreatic carcinoma in children. (See the PDQ summary on Pancreatic Cancer Treatment [Adult] for possible treatment options.)

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 Childhood Pancreatic Cancer

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

Treatment of recurrent pancreatic cancer 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 Childhood Pancreatic Cancer

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 pancreatic cancer. 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).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Pancreatic Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/pancreatic/patient/child-pancreatic-treatment-pdq. Accessed <MM/DD/YYYY>.

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