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.

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, or there may be 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.

Some of the most common symptoms include:

• Low blood sugar
• Stomach ulcers, vomiting blood, abdominal and back pain, reflux, and diarrhea from tumors that produce gastrin (gastrinomas)
• Diarrhea, dehydration, low sodium, weight changes, or high blood pressure
• Jaundice

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.

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 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:

• Physical exam
• Blood and urine tests
• Biopsy
• Magnetic resonance imaging (MRI)
• Computerized tomography scan (CT/CAT scan)
• 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 pancreatic tumor will depend on the type and location of the tumor. Your child's doctor may recommend:

• Surgery
• Radiation
• Chemotherapy

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

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.

Children with tumors of the pancreas are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center 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.

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, 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.