• Neuroendocrine Tumors

    Neuroendocrine tumors, also known as NETs, are growths that develop from specialized, hormone-producing neuroendocrine tissue distributed throughout the body.

    Neuroendocrine tumors can form in the gastrointestinal tract, lungs, pancreas, and other tissues. NETs are sometimes called carcinoid tumors, especially when they grow out of the gastrointestinal tract or lungs. Pancreatic NETs are sometimes called “islet cell” tumors and include insulinomas, glucagonomas, gastrinomas, VIPomas, and non-functioning NETs.

    Some NETs secrete hormones while others do not. Those that secrete hormones are referred to as functioning and may cause specific symptoms related to excessive production of that hormone. Most NETs are slow-growing, but some are more aggressive, growing rapidly and spreading to other parts of the body.

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

    Children with neuroendocrine tumors 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. Advanced cancers may also be treated through our Solid Tumor Center.

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

    How are neuroendocrine tumors 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.

    NETs are sometimes categorized by their location – for instance, in which part of the gastrointestinal system they are located. They are also sometimes given more specific names based on what type of hormone they produce.

    What are the causes and symptoms of neuroendocrine 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.

    Some neuroendocrine tumors result from hereditary factors. Examples of hereditary or genetic conditions that carry an increased risk for developing NETs include multiple endocrine neoplasia (MEN), familial medullary thyroid cancer (FMTC) syndrome, von Hippel-Lindau syndrome, hereditary paraganglioma-pheochromocytoma syndromes, and Carney triad.

    The symptoms of a neuroendocrine tumor may vary from child to child and can depend on where the tumor is located, what kind of neuroendocrine tumor it is, and what type of hormone it secretes. Symptoms might mimic other, more common ailments. Some of the most common symptoms may include:

    • Abdominal pain that resembles appendicitis if the tumor is located in the appendix.
    • High blood pressure, dizziness, heart palpitations, nausea, vomiting, or excessive sweating if the tumor is a pheochromocytoma or paraganglioma.
    • Abdominal pain, stomach ulcers, vomiting, diarrhea, and weight changes for NETs of the pancreas or GI tract.
    • A lump or swelling in the neck, difficulty breathing or swallowing, or hoarseness of the voice if the tumor is a medullary thyroid cancer.
    • Pain or feeling full near the site of the tumor if the tumor is large.

    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 neuroendocrine 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 neuroendocrine tumors?

    Treatment for your child's neuroendocrine tumor will depend on its type and location. Your child's doctor may recommend:

    • Surgery, involving biopsy and removal of the entire tumor and nearby tissue.
    • 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 neuroendocrine 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, he or 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 neuroendocrine tumors?

    Your child’s outlook will likely depend on where his tumor is and whether it has spread.

    Children with a neuroendocrine tumor of the appendix typically have a good prognosis after surgery. By the time they are diagnosed, neuroendocrine tumors outside the appendix have often spread or are larger. Bigger neuroendocrine tumors tend to come back more often, which we would consider in designing the treatment and follow-up plans.

    Children treated for neuroendocrine tumors 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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