Carney Triad

Carney triad is a rare condition that describes the occurrence of three kinds of endocrine tumors in the same child. The tumors comprising the triad are tumors in the gastrointestinal tract (known as gastrointestinal stromal tumors, or GIST), pulmonary chondromas, and paragangliomas. These masses grow chiefly in the stomach, the lungs, or the neuroendocrine tissues of the head, neck, and torso. Carney triad is extremely rare and is most common in females.

The symptoms of Carney triad may vary from child to child and depend on where tumors are located and what kind they are. Symptoms may mimic other, more common ailments. Some symptoms may include:

• Gastrointestinal bleeding
• Stomach pain
• Abdominal mass that can be felt on examination
• Anemia (low red blood cell count)
• Headaches
• High blood pressure

Carney triad is non-hereditary and incredibly rare, with 30 cases reported in total. Because many of these symptoms can also point to other conditions, it’s important to have your child evaluated and diagnosed 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
• Endoscopy
• Biopsy
• Magnetic resonance imaging (MRI)
• Computerized tomography scan (CT/CAT scan),
• Molecular testing

There may be other diagnostic tests 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 Carney triad will depend on a number of factors, including the type of tumors your child has and where they are located. Your child's doctor may recommend:

• Surgery
• Radiation
• Chemotherapy

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 intrathecally as a direct injection into the spinal column through a needle. Often, a combination of chemotherapy drugs is used.

Children treated for Carney triad 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 Carney triad are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through our Endocrine-Oncology Program. Advanced cancers may also be treated through our Solid Tumor Center. 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 trial provide 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. If you’re not sure which clinical trials might be right for your child, email us at clinicaltrials@danafarberbostonchildrens.org.