Neuroblastoma is the third most common cancer of childhood, occurring in approximately 600-800 children each year in the United States. Neuroblastoma is a disease in which malignant (cancer) cells form in primitive nerve tissue called “ganglions” or in cells in the adrenal glands. Normally, there are two adrenal glands present in all of us, one above each kidney. The adrenal glands typically produce important hormones that help control heart rate, blood pressure, blood sugar and the way the body reacts to stress. Patients with neuroblastoma often have elevated blood pressure or hormone problems due to the impact of the tumor on the adrenal glands. In addition to the adrenal glands, other common neuroblastoma sites include the chest or nerve tissue near the spine or spinal cord.
The most common symptom of neuroblastoma is a painless abdominal mass, usually found by a parent while changing the baby’s clothes or during a bath. Sometimes a pediatrician will discover the mass on a routine visit. Other symptoms may include fever, irritability, or limping. Neuroblastoma can be an extremely painful disease, especially if it involves the bones or bone marrow. Unfortunately, 70% of neuroblastomas have already spread to the bones or bone marrow by the time of diagnosis. Once it has spread to the bones or bone marrow, it is termed “stage 4” disease, with cure rates of only 40-50% for children over 18 months of age. Cure rates are higher for patients with lower stages (stages 1-3) of disease.
The University of Michigan C.S. Mott Children’s Hospital Pediatric Oncology Program has an active research program in pediatric neuroblastoma, with several novel protocols developed for the treatment of patients who have failed primary therapy. We provide multidisciplinary care for children with neuroblastoma, and have been a pioneer in developing diagnostic techniques and treatment therapies, with advanced clinical trials regularly available to our patients.
Cutting-Edge Options for Diagnostics and Treatment of Neuroblastoma
To diagnose neuroblastoma, a variety of tests may be ordered depending on where the disease arises. Generally, we start with an ultrasound and CT scan or an MRI of the sites involved, plus blood and urine studies. A metaiodobenzylguanidine (MIBG) scan – a procedure first discovered at the University of Michigan and now used around the world – which uses radioactive material and a scanner to find a neuroblastoma may be required, along with a PET scan and bone scan.
Our Tumor Board, made up of pediatric oncologists, surgeons, radiation oncologist, pathologists, radiologists, neurosurgeons and nuclear medicine physicians, meets to discuss each of our newly diagnosed neuroblastoma patient’s treatment plans. Together, our doctors listen to each child’s story, look closely at the scans and determine which method of treatment would be best for the individual.
Neuroblastoma has specific treatment recommendations developed by the Children’s Oncology Group, a nationwide cooperative group devoted to the study and treatment of pediatric malignancies. Based on age, staging and genetic profile, patients can be defined as having low risk, intermediate risk or high risk. Low-risk treatment can be careful observation or surgery with low-dose chemotherapy. Medium-risk treatment is generally surgery, followed by low- to medium-dose chemotherapy. High-risk treatment often involves high-dose chemotherapy, surgical resection of the primary mass, bone marrow transplant, radiation therapy and antibody therapy (can help immune system to attack cancer cells).
Advanced Research and Access to Clinical Trials for Neuroblastoma
We offer a number of neuroblastoma clinical trials for patients with relapsed or refractory disease. We have been a pioneer in using MIBG as targeted therapy for patients with refractory disease. MIBG is a form of radiation therapy directed specifically to neuroblastoma cells, sparing normal tissue from high doses of radiation. When given in low doses, MIBG is useful for imaging of neuroblastoma. When given in higher doses, MIBG can be used as a treatment for patients. We are one of a handful of pediatric institutions in the country currently utilizing high-dose MIBG therapy for patients with neuroblastoma. We recently completed a bone marrow transplant study combining MIBG as part of the transplant regimen, and are partnering with both the Children’s Oncology Group (COG) and the New Advances in Neuroblastoma Consortium (NANT) to develop new strategies for MIBG therapy.