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About Childhood Pancreatic Tumors

  • The pancreas is an organ responsible for helping the body digest food and regulate sugar. Tumors of the pancreas can develop from the organ's exocrine cells, which make enzymes to aid in digestion, or endocrine cells, which produce hormones such as insulin and glucagon that control blood sugar levels.

    Several types of pancreatic tumors can develop in children:

    • Solid pseudopapillary tumors (SPTs).
    • Pancreatoblastomas.
    • Neuroendocrine tumors (NETs). NETs in the pancreas, also known as islet cell tumors, include insulinomas, glucagonomas, gastrinomas, VIPomas, somatostatinomas, and non-functioning tumors.
    • Although pancreatic tumors are rare in children, some kinds, such as NETs, occur more commonly in children with certain inherited or genetic conditions. SPTs happen most often in girls and young women.

    Our Endocrine Oncology Program team has extensive experience treating all types of rare cancers at Dana-Farber/Boston Children's Hospital Cancer and Blood Disorders Center. We work together with you and your family from diagnosis through treatment and survivorship.

    Causes and Symptoms of Childhood Pancreatic Tumors

    It is crucial to understand that tumors often develop with no known cause. Many tumors result from a combination of genetic and environmental factors. In some cases, inherited conditions are linked to the development of pancreatic tumors, as is the case with von Hippel Lindau syndrome and multiple endocrine neoplasia (MEN) syndromes.

    The symptoms of pancreatic tumors may be different among children. They will depend on the tumor's location and if it produces hormones. Symptoms might mimic other, more common ailments.

    Pancreatic tumor symptoms may include:

    • No symptoms if a tumor is not producing hormones or feelings of pain and fullness if a non-functioning tumor grows large, pressing on nearby tissues and organs.
    • Low blood sugar, changes in behavior, seizures, or coma from tumors that secrete insulin (insulinomas).
    • Stomach ulcers, vomiting blood, abdominal and back pain, reflux, and diarrhea from tumors that produce gastrin (gastrinomas), also sometimes called Zollinger-Ellison syndrome.
    • Diarrhea, dehydration, low sodium, weight changes, or high blood pressure from tumors that make other hormones.
    • Jaundice (yellowing of the skin or eyes), itching, or light-colored stool from a tumor located in the head of the pancreas that is disrupting the bile duct.

    Because many of these symptoms can also point to other conditions, it's important to have your child evaluated by a qualified medical professional right away.

    How We Diagnose and Classify Childhood Pancreatic Tumors

    At Dana-Farber/Boston Children's, our experienced pediatric cancer specialists use advanced diagnostic tests to make an accurate diagnosis. Your child's doctor will order a combination of diagnostic tests based on your child's symptoms and health history, including imaging studies, biopsy, and blood and urine tests. We may also perform molecular testing to determine whether the tumor is linked to specific genes.

    Once the diagnosis is complete, your child's team will determine the tumor's classification. It is a way of describing where the tumor's location and whether it has spread.

    The type of pancreatic tumor your child has will help determine their prognosis and treatment. Pancreatic tumors include:

    • Insulinomas
    • Glucagonomas
    • Gastrinomas
    • VIPomas
    • Somatostatinomas
    • Non-functioning tumors
    • Solid pseudopapillary tumors
    • Pancreatoblastomas

    How We Treat Childhood Pancreatic Tumors

    We recommend treatments that are specific to the tumor's location and type. Our team develops a personalized treatment plan for your child's situation.

    Pancreatic tumor treatment options include:

    • Surgery: Surgery may involve a biopsy, removal of the entire tumor and nearby tissue, and possibly removing the adrenal gland. We use a minimally invasive approach, or laparoscopy, whenever possible.
    • Radiation therapy: This treatment uses high-energy rays from a specialized machine to damage or eliminate cancer cells and shrink tumors. We often use radiation therapy together with surgery, either before or after the removal of the cancer.
    • Chemotherapy: We may use chemotherapy, a drug treatment that aims to destroy or shrink cancer cells, before or after surgery.

      Different groups of chemotherapy drugs work in different ways. Often, we will use a combination of chemotherapy drugs. Your child may receive chemotherapy:

      • Orally, as a pill to swallow.
      • Intramuscularly, as an injection into the muscle or fat tissue.
      • Intravenously, as a direct injection into the bloodstream or IV.
      • Intrathecally, as a direct injection into the spinal column through a needle.

      While chemotherapy can be quite effective in treating certain cancers, the drugs cannot differentiate normal healthy cells from cancer cells. As a result, there can be adverse side effects during treatment. Being able to anticipate these side effects can help the care team, child, and family prepare, and in some cases prevent, these complications from occurring.

    After treatment, we recommend that your child continue care through our pediatric cancer survivorship programs. Support services include patient and family education, support groups, and counseling options.

    Research and Clinical Trials for Childhood Pancreatic Tumors

    At Dana-Farber/Boston Children's, patients benefit from the work of our clinical researchers, who are striving to understand the scientific causes of endocrine cancers. Their work results in new treatment options that may revolutionize endocrine cancer care. Find out more about our open clinical trials.

    Long-term Outcomes for Children with Pancreatic Tumors

    A patient's prognosis depends on where the tumor is and whether it has spread. With continued care from our survivorship program, your child will receive comprehensive follow-up care to catch any recurrence or other issues.

    Childhood Pancreatic Tumors Treatment Team

    Your child's care team includes a specialized team of pediatric cancer experts, including medical oncologists, surgeons, and radiation oncologists. View a complete list of the specialists in our Childhood Solid Tumor Center.