Found only in children, neuroblastoma arises in the adrenal glands, located in the abdominal area near the kidneys, and along the sympathetic nerve chain in the chest and abdomen.
Currently, one in every 285 children in the United States develops cancer before the age of 20. Progress in the development of effective new treatments and cures for childhood cancer has been spectacular during the past three decades, but progress is beginning to plateau. Most children now can be cured if they are treated at childhood cancer treatment and research centers by teams of experts in childhood cancer.
As one of the nation’s largest cancer treatment and research centers, Nationwide Children's is leading the fight against childhood cancer, determined to beat it in all its forms. The Cancer Center at Nationwide Children's Hospital provides comprehensive, multidisciplinary diagnosis, treatment and long-term management for pediatric and adolescent patients with any type of cancer. Learn more about neuroblastoma.
What is Neuroblastoma?
Neuroblastoma, a childhood cancer of the sympathetic nervous system, or the “fight or flight” response system, includes a wide variety of tumors and cancers. Neuroblastomas are tumors of the peripheral nervous system (outside the brain or spinal cord). They occur in the adrenal glands or in the paraspinal sympathetic chain (alongside the spine), with different effects on the body based on where they grow and spread (metastasis). Patients with these tumors have unique challenges specific to their disease.
Ganglioneuromas are benign tumors that can be found at any age. These benign tumors are not cancers, so they cannot spread to other parts of the body. However, they can grow in size and physically press on parts of the body, causing problems including on growth and development. These tumors usually require surgery to avoid complications, but they do not have a risk of coming back (recurrence) or metastasis.
Ganglioneuroblastomas and other low-risk neuroblastomas are usually form in children under 18 months of age. While these are cancers, they are usually small and have not spread to other parts of the body (no metastasis). These can be surgically removed (resected), but many of these may go away with time (involute) and may not require surgery. In either case, patients require monitoring for a few years (visits to clinic, labwork, and scans) but generally the cancer does not come back (recurrence). We consider the patient to be cured if the cancer does not come back within 5 years.
There is a special type of neuroblastoma in infants, called Stage MS (formerly called 4S), where the cancer cells can spread (metastasize) to the liver, skin, or bone marrow, but these children also generally have low-risk disease and can be monitored without necessarily needing treatment.
Intermediate-risk neuroblastomas are generally larger tumors that have a slightly worse chance of growing and speading, for a few different reasons. These are usually found in children between 2-5 years of age. Patients with these tumors generally require some chemotherapy and surgery to remove the main tumor. This treatment lasts about 4-8 months, and the patient spends much of that time at home with outpatient management in our clinic. They do not need radiation therapy or other aggressive treatment. Patients need to be monitored for five years after therapy, but the majority of these patients are cured after this treatment as well. There are usually few long-term effects but these patients can benefit from long-term follow-up to help with any side effects, in our survivorship clinic.
High-risk neuroblastomas are cancers that are very aggressive. They can grow in a single spot in the body (localized), but more commonly they have already spread (metastasized) by the time the child is diagnosed. Patients with these tumors, when first diagnosed, may have no symptoms or can be quite sick. Treatment for high-risk neuroblastoma is also aggressive; all hospitals in the US start treatment with chemotherapy and surgery. As part of this process, we collect a sample of the patient’s bone marrow cells early in treatment; later, we give a larger dose of chemotherapy and give them those bone marrow cells back so that they can heal (autologous bone marrow transplant). This is followed by radiation therapy, then a treatment where we make the body’s immune system fight the cancer (antibody-based immunotherapy) and biological therapy (Accutane). This treatment can take over 1 ½ years, and it will cure approximately 50-60% of these patients. In the past, patients whose cancer did not respond to treatment or had the cancers come back had few treatment options, but now we have many new ways to treat these patients. These include advanced genetic testing of tumors (genomics) that identify personalized treatment plans, infused radiation treatments (I-131 MIBG therapy) that can target multiple sites of disease, new combinations of chemotherapy, antibody therapy and specialized cell therapies, and other experimental drug studies. These treatments, all available at Nationwide Children’s Hospital, have improved outcomes for patients whose neuroblastoma has come back or hasn’t responded to standard treatment.
Some patients with neuroblastoma can also suffer from a specific neurologic complication, called opsoclonus-myoclonus-ataxia syndrome (OMAS). In these patients, their immune system actually, in the effort to fight the cancer, starts to attack the patient’s neurologic system. This can cause abnormal movements of the eyes (opsoclonus), problems with speech, unstable walking, difficulty using their hands and feet, and behavioral problems including rage attacks and disrupted sleep. This immune reaction can continue even if the tumor is fully removed. In these patients, the primary treatment is controlling the immune system, using a combination of medications. Prompt treatment is important in these cases; with good control, the patients can have very good quality of life, but when uncontrolled OMAS can impact their development long-term.
Patients with any of these types of neuroblastoma can suffer from long-term effects of their tumors or the treatment used to cure them, particularly those with high-risk disease. This can include effects on growth and development, lung and heart function, hormone levels, bone health, education, and fertility. Patients with OMAS have additional challenges neurologically and developmentally. In our survivorship clinic, we have approaches to control symptoms and help patients with any of these conditions, allowing them to lead full and long lives.
What are the Treatment Options for Neuroblastoma?
The types of treatment used most often to treat childhood cancer are surgery, chemotherapy, radiation therapy, and bone marrow transplantation. The goal of treatment is to destroy the cancer cells. Your child may have one kind of treatment or a combination of treatments and they may have to have a variety of tests and procedures during treatment. Their treatment plan depends on the type of cancer, stage of disease and many other factors. Before treatment starts, your doctor will discuss the treatment plan with you including expected benefits, risks and side effects. Nationwide Children's also participates in clinical trials.
The Blood and Cancer Center at Nationwide Children's Hospital is one of the largest pediatric cancer centers in the country and a member of the Children’s Oncology Group (COG), which ensures that your child will get the highest quality care available anywhere in the country. Our program is uniquely family-centered, with patients benefiting from a multi-disciplinary clinical team and state-of-the-art facility.
Surgery is a local therapy that involves the surgical removal of all or part of the cancer. Often, surgery is used with chemotherapy and/or radiation therapy. The type of operation will depend on the location of the main tumor, its size and other individual factors.
Chemotherapy is the treatment of cancer with “anti-cancer” drugs to destroy cancer cells throughout the body. Chemotherapy drugs slow or stop cancer cells from growing and making more abnormal cells. These drugs also may affect normal healthy cells, but healthy cells can repair and return to normal.
Radiation Therapy is the treatment of cancer and other diseases with high-energy rays to damage or destroy cancer cells. Radiation damages or destroys the cells in the area being treated making it impossible for the cancers cells to continue to grow and multiply. Most radiotherapy is delivered from the outside of the body (external beam radiotherapy) usually in the form of high energy X-rays. Radiation therapy can damage normal cells as well as cancer cells. When this happens, side effects occur. If your child is receiving radiation therapy, you will receive more information from your doctors and nurses.
A Bone Marrow Transplant (BMT) replaces diseased bone marrow with healthy bone marrow. Bone marrow is the soft material inside the bones of the body that makes blood cells. In an autologous bone marrow transplant, the patient’s own bone marrow is treated and reinfused (put back into the patient). In an allogenic bone marrow transplant, healthy marrow comes from a donor.
Within 5 days of her neuroblastoma diagnosis, Amelia had surgery at Nationwide Children’s to remove the tumor that was resting on her spine and aorta. With less than a 5% chance that the tumor would return, there was no chemotherapy used.
Her cancer journey didn't end there, but now she's in remission.
Nationwide Children's Hospital has the largest and most advanced pediatric cancer program in Ohio – and one of the largest in the nation. In 2004, more than 1,000 children were treated at Nationwide Children's – the highest number of pediatric oncology inpatient visits in the state. More than 75 percent of children who receive a diagnosis of cancer in 2005 will be cancer-free five years later, but much work remains to be done to find cures for cancers for which there is no effective pro-tocol. Nationwide Children's Research Institute ranks in the top 10 of free-standing pediatric research centers based on National Institutes of Health funding.
Tests and Procedures
Depending on your child’s diagnosis, he or she may need to have tests or procedures done periodically through their treatment. These may be done to see how your child is responding to treatment or to keep a watch on side effects.
Click on the links below to learn more about specific tests and procedures:
You or your child may be asked to participate in a clinical trial. Your participation in this research study is voluntary.
A clinical trial is a research study in which physicians find ways to improve cancer treatment. The goals of these studies are to answer scientific questions about preventing, diagnosing and treating cancer.
A clinical trial for cancer treatment occurs in three phases.
Phase I – This is a first study done on a drug to test its safeness, to determine the right dose, and to determine when and how to give the drug. These studies usually are limited to a small number of patients.
Phase II – As in Phase I, this phase also tests the safety of a drug, but the trial also tests how well the drug works to treat different types of cancers.
Phase III – This phase of a clinical trial studies various drugs, usually in combination, against standard therapy. These studies enroll large numbers of patients at many different cancer centers.
Cancer treatment has improved greatly over the past several decades due to the use of clinical trials. Children’s Hospital does Phase I, Phase II and Phase III clinical trials. If you are asked to participate in a clinical trial, the physician will review the study and consent form with you and answer your questions. You will receive a pamphlet with more detailed information about clinical trials to help you make a decision.
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