• Tumors of the Pancreas

    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 different types of pancreatic tumors can develop in children, such as solid pseudopapillary tumors (SPTs), pancreatoblastomas, and neuroendocrine tumors (NETs). NETs in the pancreas, also known as islet cell tumors, include insulinomas, glucagonomas, gastrinomas, VIPomas, somatostatinomas, and nonfunctioning 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 are most often seen in girls and young women.

    Pancreatic Tumor Treatment at Dana-Farber/Boston Children's

    Children with tumors of the pancreas are treated at Dana-Farber/Boston Children's through our Endocrine-Oncology Program. Our integrated pediatric oncology service offers—in one specialized program—the combined expertise of a leading cancer center and a premier children’s hospital. We build a team to treat your child consisting of oncologists, endocrinologists, genetic counselors, and surgeons.

    Continue reading for more information about tumors of the pancreas or visit the Endocrine-Oncology Program page to learn more about our expertise or meet our treatment team.

    How are tumors of the pancreas classified?

    Once diagnosis is complete, your child’s team will likely determine the tumor’s stage or classification, a way of describing where the tumor is located and whether it has spread.

    The type of pancreatic tumor your child has will help determine his prognosis and treatment. Types of tumors of the pancreas include:

    • Insulinomas
    • Glucagonomas
    • Gastrinomas
    • VIPomas
    • Somatostatinomas
    • Nonfunctioning tumors
    • Solid pseudopapillary tumors
    • Pancreatoblastomas

    What are the causes and symptoms of pancreatic tumors?

    As a parent, you undoubtedly want to know what may have caused your child’s tumor. It is important to understand that often tumors emerge with no known cause. Many result from a mingling 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 syndromes.

    The symptoms of pancreatic tumors may vary from child to child and depend on where the tumor is located and whether it produces hormones. Symptoms might mimic other, more common ailments. Some of the most common symptoms 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). This is 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 are pancreatic tumors diagnosed?

    The first step in treating your child is forming an accurate and complete diagnosis. Your child’s physician may order a number of different tests including:

    • A physical exam and complete medical history.
    • Blood and urine tests.
    • A biopsy, a tissue sample taken from the tumor. The tumor's appearance under a microscope helps doctors to make a diagnosis so the appropriate treatments can be recommended.
    • Magnetic resonance imaging (MRI), a diagnostic procedure that produces detailed images of the area where the tumor is located. An MRI uses a combination of large magnets, radiofrequencies, and a computer to analyze organs and structures within the body.
    • A computerized tomography scan (CT/CAT scan), an imaging technique that provides more detailed pictures than X-rays.
    • Molecular testing to determine whether the tumor is linked to specific genes.

    There may be other diagnostic tests that your doctor will discuss with you depending on your child's individual situation. After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then, we will meet with you and your family to discuss the results and outline the best possible treatment options.

    What are the treatments for pancreatic tumors?

    Treatment for your child's pancreatic tumor will depend on the type and location of the tumor. Your child's doctor may recommend:

    • Surgery, involving biopsy and removal of the entire tumor and nearby tissue. A minimally invasive approach, such as laparoscopy, is possible for many tumors.
    • Radiation, the use of high-energy rays from a specialized machine to damage or kill cancer cells and shrink tumors. This is often used together with surgery, either before or after removal of the tumor.
    • Chemotherapy, a drug treatment that aims to destroy or shrink cancer cells, may be given before or after surgery.
      • Different groups of chemotherapy drugs work in different ways. 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; or intrathecally, as a direct injection into the spinal column through a needle. Often, a combination of chemotherapy drugs is used.
      • 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, if at all possible.

    What is the latest research on pancreatic tumors?

    Children who are treated through our Endocrine-Oncology Program benefit from the work of our basic and clinical researchers, who are striving to understand the scientific causes of endocrine cancers. Their work can result in the introduction of new treatment options. We are a world leader in translational research, bringing laboratory advances to the bedside and into doctors’ offices as quickly as possible.

    Clinical Trials

    Clinical trials, or research studies evaluating new treatment approaches, are a major offering at Dana-Farber/Boston Children’s. For many children with rare or hard-to-treat conditions, clinical trials provide new options.

    It’s possible that your child will be eligible to participate in one of our clinical trials. In addition to launching our own clinical trials, we also offer trials available through collaborative groups such as the Children's Oncology Group (COG). If your child has a progressive or recurrent tumor, she may be eligible for a number of experimental therapies available through these groups or from one of our independent clinical investigators.

     

    What is the long-term outlook for pancreatic tumors?

    Your child’s prognosis will depend on the type of tumor he or she has, the specific location of the tumor in the pancreas, whether it is benign or malignant, and whether it has spread.

    Children treated for tumors of the pancreas should visit a survivorship clinic yearly. Through the David B. Perini, Jr. Quality of Life Clinic, our cancer survivorship clinic, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team. In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists, or alternative/complementary therapy specialists. We also offer patient and family education, psychosocial assessment, genetic counseling, reproductive counseling, and opportunities to speak with other childhood cancer survivors.

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