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Neuroblastoma
The Cancer Center at The Children's Hospital of Philadelphia has a multidisciplinary team of highly experienced and compassionate professionals who will provide expert management of your child's cancer. In addition, Children's Hospital researchers are actively involved in researching and developing new therapies to treat neuroblastoma.
What is neuroblastoma?
Neuroblastoma is a tumor of nerve tissue that develops in infants and children and can occur in many areas of the body. It develops from the tissues that form the sympathetic nervous system (which controls body functions, such as heart rate and blood pressure, digestion and levels of certain hormones). It most commonly begins in the abdomen in the tissues of the adrenal gland, but it may also occur in other areas. It can spread to the lymph nodes, liver, bones and bone marrow. The cause of the tumor is unknown.
Who is diagnosed with neuroblastoma?
Neuroblastoma is typically found in babies or young children. The median age at diagnosis is two years. It is found slightly more often in boys than in girls. In the United States, about 600 to 800 cases are diagnosed each year.
What are the signs and symptoms of neuroblastoma?
Signs and symptoms will vary depending on the site of the primary tumor or the extent that it has spread (metastasized). The most common primary site is the abdomen. Other primary sites include the chest, neck and pelvis. Neuroblastoma can spread to other areas of the body. If your child has a primary tumor in the abdomen, you may notice bellyaches, weight loss or a bigger abdomen. Some children only complain of general aches and pains.
How is neuroblastoma diagnosed?
Your child will need to have a series of tests to help us learn the extent of the disease. Some of the neuroblastoma tests include:
- Urine VMA/HVA test. This test will determine the amount of a substance called catecholamines in the urine; the tumor secretes and produces catecholamines.
- X-rays. Chest and skeletal X-rays may be performed.
- Bone scan. Dye is injected into a vein and an X-ray is taken to see if the disease has spread to the bones.
- CT Scan (Computerized Tomography). A type of X-ray, this test is done to get pictures of the abdomen and/or pelvis to look for the spread or extent of the disease.
- Bone marrow aspirate and biopsy. Bone marrow is removed from the bone (usually the hip bone) either by aspiration (suctioning a small amount through a hollow needle) or by biopsy (cutting out a small piece of bone marrow).
- MIBG scan. A small amount of radioactive iodine is injected and an area of the body is viewed through pictures taken 24, 48, and sometimes 72 hours later. Your child will need to take iodine drops for three to four days before an MIBG scan.
- Surgical biopsy. This test allows us to collect a small sample of the tumor. It is done in the operating room under general anesthesia. After the surgery, laboratory tests are done on the tumor sample.
How is neuroblastoma treated?
We start by determining whether the disease risk is low, intermediate or high.
- Low-risk disease. If your child is under a year of age at diagnosis, or has small, easily removed tumors, the child may only need a simple surgical procedure or careful follow-up.
- Intermediate-risk disease. If your child has a larger tumor, we will probably treat the child with surgery and four to eight months of chemotherapy. Often, patients with intermediate-risk disease do not need radiation treatments.
- High-risk disease. More than half of neuroblastoma patients have high-risk disease, either because the tumor has spread to other parts of the body or because test results show high-risk features. If your child has high-risk disease, treatment includes five months of chemotherapy, surgery, radiation therapy, stem cell transplants and other treatments. These very intense treatments have improved the cure rate of this complex disease.
Newly diagnosed patients are generally treated according to Children's Oncology Group protocols. Patients with the aggressive form of the disease may be treated according to institutional protocols under the direction of Stephan A. Grupp, MD, PhD.
A team of investigators is dedicated to providing options for families of children with relapsed or refractory disease through an evolving portfolio of clinical trials, including those through the New Approaches to Neuroblastoma Therapy consortium. Consultation with the Neuroblastoma Developmental Therapeutics Team (John M. Maris, MD; Yael P. Mosse, MD; Pat Brophy, CRNP) is required to determine eligibility and suitability for any trial. After consultation, the team can develop a recommendation to be provided to the referring physician.
Late effects/cancer survivorship
Some children treated for cancer develop complications years later. Our Cancer Survivorship Program provides information about the potential long-term effects of the specific treatment your child received, including ways of monitoring and treating these effects.
Neuroblastoma research news
View the latest news in neuroblastoma research.
Reviewed by: John M. Maris, MD, The Children's Hospital of Philadelphia
Date: May 2005
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