Neuroendocrine Tumors (NETs)

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

These 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.

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
• High blood pressure, dizziness, heart palpitations, nausea, vomiting, or excessive
• Stomach ulcers, vomiting, diarrhea, or weight changes
• Lump or swelling in the neck
• 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 for diagnosis by a qualified medical professional right away.

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 first step in treating your child is checking symptoms and forming a complete diagnosis.

Your child’s physician may order a number of different tests including:

• Physical exam and complete medical history
• Blood and urine tests
• Biopsy
• Magnetic resonance imaging (MRI)
• Computerized tomography scan (CT/CAT scan)
• Molecular genetic testing

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.

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
• Chemotherapy

Different groups of chemotherapy drugs work in different ways. Your child may receive chemotherapy orally, intramuscularly, intravenously, or intrathecally. 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.

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.

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

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.