Childhood Pancreatic Cancer

Certain types of pancreatic cancer are more commonly seen in children than in adults. Types of pancreatic cancers and tumors seen in children include:

Solid pseudopapillary tumor of the pancreas

This is the most common type of pancreatic tumor in children. It usually affects older adolescent and young adult females. Solid pseudopapillary tumor of the pancreas is unlikely to spread to other parts of the body and the prognosis is generally good.

Pancreatoblastoma

Pancreatoblastoma usually occurs in children aged 10 years or younger. Children with certain genetic syndromes like Beckwith-Wiedemann syndrome and familial adenomatous polyposis (FAP) syndrome have an increased risk of developing pancreatoblastoma. These tumors may produce hormones such as adrenocorticotropic hormone (ACTH) or antidiuretic hormone (ADH). The prognosis for children with pancreatoblastoma is generally good. 

Pancreatic neuroendocrine tumor (islet cell tumor)

Pancreatic neuroendocrine tumors (NETs), also called islet cell tumors, are rare in children and can be benign (not cancer) or malignant (cancerous). Children with multiple endocrine neoplasia type 1 (MEN1) syndrome have an increased risk of developing pancreatic NETs. The prognosis depends on the type of pancreatic NET your child has. Learn more about Pancreatic Neuroendocrine Tumors and MEN1 Syndrome.

Pancreatic carcinoma

Pancreatic carcinoma is very rare in children, but it is the most common type of pancreatic cancer in adults. The two types of pancreatic carcinoma are ductal adenocarcinoma, the most common tumor type in adults, and acinar cell carcinoma. Learn more about these types of pancreatic cancer at What Is Pancreatic Cancer?

Childhood pancreatic cancer is caused by certain changes to the way pancreas cells function, especially how they grow and divide into new cells. Often, the exact cause of the cell changes is unknown. Learn more about how cancer develops at What Is Cancer?

A risk factor is anything that increases the chance of getting a disease. Not every child with a risk factor will develop pancreatic cancer, and it will develop in some children who don’t have a known risk factor. 

Childhood pancreatic cancer may be part of a growth disorder or inherited cancer syndrome. Inherited cancer syndromes are caused by changes in certain genes passed from parents to children. The following inherited cancer syndromes or growth disorders increase a child’s risk of pancreatic cancer:

• familial adenomatous polyposis (FAP) syndrome
• familial atypical multiple mole melanoma (FAMM) syndrome
• Peutz-Jeghers Syndrome (PJS)
• Li-Fraumeni Syndrome (LFS)
• multiple endocrine neoplasia type 1 (MEN1) syndrome
• von Hippel-Lindau syndrome
• Beckwith-Wiedemann syndrome

It may not be clear from the family history whether a child with pancreatic cancer has an inherited condition that increased their risk. Genetic testing may help explain why a child develops a rare cancer or a cancer that is usually seen in adults, such as pancreatic cancer. Genetic counselors and other specially trained health professionals can discuss and evaluate your child’s diagnosis and your family’s medical history to help you understand: 

• your options for gene testing
• the risk of other cancers for your child
• the risk of pancreatic cancer and other cancers for your child’s siblings
• the risks and benefits of learning genetic information

Genetic counselors can also help you cope with your child’s genetic test results, including how to discuss the results with family members. They can also advise about whether other members of your family should receive genetic testing.

Often, pancreatic cancer does not have early signs or symptoms in children. When they do occur, general signs and symptoms may include:

• feeling very tired
• weight loss for no known reason
• loss of appetite
• stomach discomfort
• lump in abdomen
• jaundice, which includes symptoms such as:
  • yellowing of the skin and whites of the eyes
  • light-colored stools
  • dark urine
  • itching

Pancreatoblastoma may produce the hormones adrenocorticotropic hormone (ACTH) or antidiuretic hormone (ADH). If the tumor makes these hormones, symptoms may include:

• watery diarrhea
• dehydration
• low sodium levels in the blood, which can cause 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

Some types of pancreatic neuroendocrine tumors (NETs) produce other hormones that may cause signs and symptoms. The signs and symptoms depend on the type of hormone being made. Learn more at Pancreatic Neuroendocrine Tumor Signs and Symptoms.

Check with your child’s doctor if you see any of these problems in your child. Conditions other than pancreatic cancer may also cause these signs and symptoms.

Pancreatic cancer is usually diagnosed with tests that make pictures of the pancreas and the area around it. Some of these tests are also used to find out if cancer cells have spread beyond the pancreas, a process called staging. 

Tests and procedures to find, diagnose, and stage pancreatic cancer are usually done at the same time. In children, there is no formal staging system for pancreatic cancer, but the results of these tests will help your child’s doctor plan treatment without assigning a stage to the cancer.

In addition to asking about your child’s personal and family medical history and doing a physical exam, the doctor may use the following tests and procedures to diagnose pancreatic cancer:

Laboratory tests

• Blood chemistry study uses a blood sample to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual amount of a substance could be a sign of disease.

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• Tumor marker test uses a blood sample to measure the amounts of certain substances, called tumor markers, released into the blood by tumor cells in the body. Increased levels of certain tumor markers have been linked to specific types of cancer.

Imaging tests

• 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. A CT scan is the most commonly performed imaging test and is also called computed tomography, computerized tomography, or computerized axial tomography. Learn more at Computed Tomography (CT) Scans and Cancer.
• MRI (magnetic resonance imaging) uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas in the body, such as the chest, abdomen, and pelvis. This procedure is also called nuclear magnetic resonance imaging (NMRI).

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• PET scan (positron emission tomography scan) uses a small amount of radioactive sugar (also called radioactive glucose) that is injected into a vein. The PET scanner rotates around the body and makes a picture of where sugar is being used by the body. Cancer cells show up brighter in the pictures because they are more active and take up more sugar than normal cells do. When this procedure is done at the same time as a CT scan or an MRI, it is called a PET-CT scan or a PET-MRI.
• Ultrasound uses high-energy sound waves (ultrasound) that bounce off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. Abdominal ultrasound, endoscopic ultrasound, or intraoperative ultrasound may be used to look for pancreatic cancer in children.
• Endoscopic retrograde cholangiopancreatography (ERCP) uses an endoscope and x-rays to look at the ducts 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, which blocks or slows the flow of bile and causes jaundice. ERCP can find a blockage and relieve it by placing a stent during the procedure.
• Percutaneous transhepatic cholangiography (PTC) uses x-rays and a contrast to look at hepatic and common bile ducts. Sometimes pancreatic cancer causes these ducts to become blocked. PTC can find a blockage in the bile ducts and relieve it by placing a plastic tube that drains bile to a bag on the outside of the body. 

Biopsy

Biopsy is the removal of a sample of cells or a small piece of tissue from the tumor so that a pathologist can view it under a microscope to check for cancer. Cells may be removed using a fine or wide needle inserted into the pancreas during an x-ray or ultrasound. Tissue may also be removed during a minimally invasive surgery called laparoscopy.

Molecular testing

Molecular testing checks for certain genes, proteins, or other molecules in a sample of tissue, blood, or bone marrow. A molecular test may be used to help plan treatment, find out how well treatment is working, or make a prognosis. 

Tests for pancreatic neuroendocrine tumor (islet cell tumor)

Other tests may be used for diagnosing pancreatic neuroendocrine tumors (NETs):

• Blood hormone level tests check the blood for increased levels of certain hormones. Because some pancreatic NETs produce hormones, these tests can help diagnose pancreatic NETs and determine the type.
• Somatostatin receptor scintigraphy is a radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein. The radioactive octreotide attaches to the tumor cells in the NETs, and a special camera that detects radioactivity shows where the tumors are in the body. This procedure is also called octreotide scan.
• Angiogram looks at blood vessels and the flow of blood. A contrast dye is injected into the blood vessel. As the contrast dye moves through the blood vessel, x-rays are taken to see if there are any abnormalities that could be signs of a tumor. Angiograms can help diagnose pancreatic NETs.
• Bone scan checks 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 bones with cancer and is detected by a scanner. Bone scans can help diagnose pancreatic NETs.

You may want to get a second opinion to confirm your child’s 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. This doctor may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child’s 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 or hospital that can provide a second opinion. For questions you might want to ask at your child’s appointments, visit Questions to Ask Your Doctor About Cancer.

There are different types of treatment for children and adolescents with pancreatic 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, the extent of the cancer, 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 pancreatic 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:

• pediatrician
• pediatric surgeon
• pathologist
• endocrinologist
• pediatric nurse specialist
• social worker
• rehabilitation specialist
• psychologist
• child-life specialist
• fertility specialist
• nutritionist

Your child’s treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. If the cancer occurs as part of an inherited cancer syndrome, the treatment plan will also consider other medical conditions caused by the syndrome, such as other cancers or polyps, which may affect the type and extent of surgery. 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, visit Children with Cancer: A Guide for Parents.

Types of treatment your child might have include:

Surgery

Surgery to remove the tumor is used to treat most types of pancreatic cancer. The type of surgery depends on the type, location and size of the tumor. Types of surgery include:

• Enucleation removes the tumor only. This may be done when tumor occurs in one place in the pancreas.
• Distal pancreatectomy is used for tumors in the body and tail of the pancreas. It involves removing part of the body and tail of the pancreas and sometimes the spleen.
• Whipple procedure, also called pancreaticoduodenectomy, removes tumors in the head of the pancreas. It involves removing the head of the pancreas, the gallbladder, part of the small intestine, part of the bile duct, and part of the stomach. Enough of the pancreas is left to produce digestive juices and insulin.

Learn more about Surgery to Treat Cancer.

Chemotherapy

Chemotherapy uses drugs to kill cancer cells or stop them from growing. It can be used alone or in combination with other treatments. 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 used alone or in combination to treat pancreatic cancer in children may include:

• cisplatin
• doxorubicin
• 5-fluorouracil (5-FU)
• vincristine
• cyclophosphamide
• gemcitabine

Chemotherapy regimens given to adults may also be used. Learn more about these regimens at Pancreatic Cancer Treatment.

Learn more about how chemotherapy works, how it is given, common side effects, and more at Chemotherapy to Treat Cancer.

Radiation therapy

Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Your child’s doctor may recommend two different types of radiation therapy, depending on the type of tumor:

• External radiation therapy and stereotactic radiation therapy, which is a more focused way of delivering radiation. These therapies use a machine outside the body to send radiation toward the area of the body with cancer. This may be used to shrink the tumor if it cannot initially be removed with surgery. Learn more about External Beam Radiation Therapy for Cancer.
• Internal radiation therapy. Peptide receptor radionuclide therapy (PRRT) is a type of internal radiation therapy that may be used to treat pancreatic NETs. It targets cancer cells using a radioactive chemical linked to a peptide (small protein). When this radioactive peptide is injected into the body, it binds to a specific receptor found on some pancreatic NETs. The radioactive peptide builds up in these cells and helps kill the cancer cells without harming normal cells. Lutetium Lu 177-Dotatate is a type of PRRT that may be used to treat pancreatic NETs in children.

Children with cancer that occurs because of certain inherited cancer syndromes, like Li-Fraumeni syndrome, cannot receive radiation therapy. Learn more about External Beam Radiation Therapy for Cancer and Radiation Therapy Side Effects.

Immunotherapy

Immunotherapy helps a person’s immune system fight cancer. Immunotherapy may be used to treat a small number of pancreatic cancers that have certain changes in their DNA, such as high microsatellite instability (MSI-H), deficient DNA mismatch repair (dMMR), or high tumor mutational burden (TMB-H). Pembrolizumab and dostarlimab are immunotherapies used to treat these types of pancreatic cancer when other treatments haven’t worked, when the cancer has spread to other parts of the body, or when the tumor cannot be removed by surgery.

Your child’s doctor will suggest biomarker tests to help predict your child’s response to immunotherapy. Learn more about Biomarker Testing for Cancer.

Learn more about Immunotherapy to Treat Cancer and Immunotherapy Side Effects.

Targeted therapy

Targeted therapy uses drugs or other substances to block the action of specific enzymes, proteins, or other molecules involved in the growth and spread of cancer cells. Everolimus is a targeted therapy drug that may be used to treat pancreatic NETs in children.

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 at ClinicalTrials.gov.

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

To learn more about the treatments listed here, visit the section, Types of treatment for childhood pancreatic cancer.

Treatment of solid pseudopapillary tumor of the pancreas

Treatment for solid pseudopapillary tumor of the pancreas in children may include:

• Surgery to remove the tumor, which is the most common treatment.
• Chemotherapy for tumors that cannot be removed by surgery or have spread to other parts of the body.

Treatment of pancreatoblastoma

Treatment for pancreatoblastoma in children may include:

• Surgery to remove the tumor.
• Chemotherapy may be given to shrink the tumor before surgery.
• Chemotherapy may be given after surgery for tumors that could not be fully removed by surgery, tumors that have spread to other parts of the body, and tumors that have come back after initial treatment.
• External beam radiation therapy may be given for tumors that could not be fully removed by surgery.

Treatment of pancreatic neuroendocrine tumor (islet cell tumor)

Treatment for pancreatic neuroendocrine tumor (NET) in children depends on the type of NET your child has. Generally, treatment may include:

• Drugs to treat symptoms caused by hormones made by the tumor.
• Surgery to remove the tumor.
• Chemotherapy, targeted therapy, internal radiation therapy, or a combination of these may be given for pancreatic NETs that cannot be removed by surgery or that have spread to other parts of the body.

Learn more about Pancreatic Neuroendocrine Tumors.

Treatment of pancreatic carcinoma in children

Pancreatic carcinoma, including acinar cell and pancreatic ductal adenocarcinoma, is very rare in children. Possible treatment options for children would include those for adults. Learn more about Pancreatic Cancer Treatment in adults.

Cancer treatments can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child’s treatment team about which side effects to look for and ways to manage them.

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.

Learn more about Late Effects of Treatment for Childhood Cancer.

As your child goes through treatment, they will have follow-up tests or check-ups. Some of the tests that were done to diagnose the cancer such as tumor marker and imaging tests, 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). To learn more about follow-up tests, visit Tests to diagnose childhood pancreatic cancer.

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. Learn more at Support for Families: Childhood Cancer and in the booklet Children with Cancer: A Guide for Parents.