Childhood Pancreatic Cancer Treatment (PDQ®): Treatment - Patient Information [NCI]

General Information About Childhood 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.
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:

• Endocrine pancreas cells make hormones, such as insulin and glucagon, that help control blood sugar levels. Both of these hormones help the body use and store the energy it gets from food. Endocrine pancreas cells cluster together in many small groups (islets) throughout the pancreas. Endocrine pancreas cells are also called islet cells or islets of Langerhans.
• Exocrine pancreas cells make enzymes that are released into the small intestine to help the body digest food. Most of the pancreas is made of ducts and small sacs at the end of the ducts that are lined with exocrine cells.

There are four types of pancreatic cancer in children.

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

• Solid pseudopapillary tumor of the pancreas. This is the most common type of pancreatic tumor in children. It most commonly affects females that are older adolescents and young adults. These slow-growing tumors have both cyst -like and solid parts. Solid pseudopapillary tumor of the pancreas is unlikely to spread to other parts of the body and the prognosis is very good. Occasionally, the tumor may spread to the liver, lung, or lymph nodes.
• Pancreatoblastoma. It usually occurs in children aged 10 years or younger. Children with Beckwith-Wiedemann syndrome and familial adenomatous polyposis (FAP) syndrome have an increased risk of developing pancreatoblastoma. These slow-growing tumors often make the tumor marker alpha-fetoprotein. These tumors may also make adrenocorticotropic hormone (ACTH) and antidiuretic hormone (ADH). Pancreatoblastoma may spread to the liver, lung, or lymph nodes. The prognosis for children with pancreatoblastoma is good.
• Islet cell tumors. These tumors are not common in children and can be benign or malignant. Islet cell tumors may occur in children with multiple endocrine neoplasia type 1 (MEN1) syndrome. (See the PDQ summary on Childhood Multiple Endocrine Neoplasia [MEN] Syndromes Treatment for more information.) The most common types of islet cell tumors are insulinomas and gastrinomas. Other types of islet cell tumors are ACTHoma and VIPoma. These tumors may make hormones, such as insulin, gastrin, ACTH, or ADH. When too much of a hormone is made, signs and symptoms of disease occur. These tumors are also called pancreatic neuroendocrine tumors (pancreatic NETs).
• Pancreatic carcinoma. Pancreatic carcinoma is very rare in children. The two types of pancreatic carcinoma are acinar cell carcinoma and ductal adenocarcinoma.

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.
  
  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.
  
  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

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.

Treatment Option Overview

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:

• Pediatrician.
• Pediatric surgeon.
• Pathologist.
• Endocrinologist.
• Pediatric nurse specialist.
• Social worker.
• Rehabilitation specialist.
• Psychologist.
• Child-life specialist.

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.

• mTOR inhibitor therapy: mTOR inhibitor drugs stop the protein that helps cells divide and survive. This type of drug is used to treat islet cell tumors and is being studied to treat pancreatic carcinoma.

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:

• Surgery to remove the tumor.
• Chemotherapy and targeted therapy (mTOR inhibitor therapy) for tumors that cannot be removed by surgery or that have spread to other parts of the body.

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:

• A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.

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

For more information from the National Cancer Institute about pancreatic cancer, see the following:

• Pancreatic Cancer Home Page
• Computed Tomography (CT) Scans and Cancer
• Targeted Cancer Therapies

For more childhood cancer information and other general cancer resources, visit:

• About Cancer
• Childhood Cancers
• CureSearch for Children's Cancer
• Late Effects of Treatment for Childhood Cancer
• Adolescents and Young Adults with Cancer
• Children with Cancer: A Guide for Parents
• Cancer in Children and Adolescents
• Staging
• Coping with Cancer
• Questions to Ask Your Doctor about Cancer
• For Survivors and Caregivers

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).

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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: https://www.cancer.gov/types/pancreatic/patient/child-pancreatic-treatment-pdq. Accessed <MM/DD/YYYY>.

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Last Revised: 2021-02-19

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