Carcinoid Tumors

As a parent, you undoubtedly want to know what may have caused your child’s tumor. Some carcinoid tumors are linked to inherited conditions, such as MEN1. However, most carcinoid tumors emerge with no known cause.

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

• Abdominal pain due to appendicitis. Most cases of appendicitis, however, are not related to carcinoid tumors.
• In rare cases, children may develop carcinoid syndrome, with symptoms such as redness in the face and neck, rapid heartbeat, difficulty breathing, sudden drop in blood pressure, and diarrhea. These symptoms are caused by hormones produced by the tumor and rarely occur except in advanced cases.

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.

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.

Treatment for your child's carcinoid tumor will depend on its location and whether it has spread. 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.

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.

• Get answers to common questions about clinical trials for cancer and blood disorders.
• Contact us: If you’re not sure which clinical trials might be right for your child, email us at clinicaltrials@danafarberbostonchildrens.org. We can help you navigate your options.

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

For tumors growing in the appendix, often an appendectomy is the only thing needed to completely remove the tumor. In this situation, a child’s prognosis is excellent. Larger tumors in other locations or those that have spread throughout the body are more difficult to treat and may require surgery, chemotherapy, radiotherapy, or other therapies or interventions.

Children treated for carcinoid 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.