Children’s National Medical Center’s team of doctors, nurses, researchers social workers, a psychologist, an art therapist, a child life specialist, a nutritionist, and a chaplain, work collaboratively to provide the best care for patients with cancer.
Children’s patients have access to the most current therapies in treating pediatric cancer through clinical trials. In addition, the Patient and Family Support Program provides comprehensive mental health and psychosocial services and helpful resources for all patients treated in the Center for Cancer and Blood Disorders, as well as patients’ parents and siblings.
What is neuroblastoma?
Neuroblastoma is one of the most common types of solid tumors found in children and accounts for approximately 7 to 10 percent of all childhood cancers. In the United States, approximately 650 children are diagnosed with neuroblastoma each year. It is often present at birth, but not detected until the tumor begins to grow and compress the surrounding organs. Most children affected by neuroblastoma have been diagnosed before the age of 5. In rare cases, neuroblastoma can be detected before birth by a fetal ultrasound. It is the most common tumor found in children younger than 1 year of age. For unknown reasons, it occurs slightly more often in males than in females.
Neuroblastoma is a cancerous tumor that begins in nerve tissue of infants and very young children. The abnormal cells are often found in the nerve tissue that is present in the unborn baby and later develops into a detectable tumor. Neuroblastoma is rare in children older than 10 years of age; however, it does occur occasionally in adults.
The tumor usually begins in the tissues of the adrenal gland found in the abdomen, but may also begin in nerve tissue in the neck, chest, and/or pelvis. The adrenal glands are positioned on top of the kidneys. These glands secrete hormones and other important substances that are required for normal functions in the body such as the nervous system.
Neuroblastoma cancer cells can spread (metastasize) quickly to other areas of the body (i.e., lymph nodes, liver, lungs, bones, central nervous system, and bone marrow). Approximately 70 percent of all children diagnosed with neuroblastoma will have some metastatic disease.
Back to Top
What are the causes of neuroblastoma?
Most neuroblastoma cells have genetic abnormalities involving chromosome #1 where a deletion or rearrangement is found on the short arm of this chromosome. The chromosome abnormality, in turn, causes amplification of an oncogene called n-myc, even though this gene is not located on chromosome #1. The amplification of n-myc causes uncontrolled cell growth. A variety of other chromosome abnormalities may also be present in neuroblastoma.
As many as 20 percent of neuroblastoma cases are caused by an inherited mutation, followed by a second mutation occurring after birth, which together initiate uncontrolled cell growth. The remainder of the cases occur from two acquired mutations after birth. Because the tumor occurs very early in childhood, it is doubtful that any environmental exposures the child has incurred could be linked to the development of the tumor.
Neuroblastoma is more common in children born with fetal hydantoin syndrome, neurofibromatosis, and Beckwith-Wiedemann syndrome. The exact relationship between these conditions and the disease are not known.
The chance for neuroblastoma to be present in a future sibling of the patient is approximately 1 percent. If more than one child has neuroblastoma, the chance for reoccurrence increases.
Research is being conducted to determine if maternal exposure to any toxic substances, environmental pollutions or radiation during pregnancy could have any link to the child developing neuroblastoma.
Back to Top
What are the symptoms of neuroblastoma?
The following are the most common symptoms of neuroblastoma. However, each child may experience symptoms differently. The symptoms of neuroblastoma vary greatly depending on size, location, and spread of the tumor. Symptoms may include:
The symptoms of neuroblastoma may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
- Abdominal mass, either felt during an examination or seen as swollen abdomen.
- Swelling and bruising around the eyes and uncontrolled eye movement.
- Changes in urination caused by the tumor compressing the kidney or bladder.
- Pain, limping, paralysis, or weakness from bone marrow involvement.
- Diarrhea caused by a substance produced by the tumor (vasoactive intestinal peptide, or VIP).
- High blood pressure and increased heart rate may occur depending on location of tumor and the organs the tumor compresses.
- Weight loss and/or decreased appetite.
- Fatigue (feeling tired or weak).
- Difficulty walking.
Back to Top
How is neuroblastoma diagnosed?
In addition to a complete medical and physical examination, diagnostic procedures for neuroblastoma may include:
Diagnosing neuroblastoma also involves staging and classifying the disease, which determines treatment options and prognosis. Staging is the process of determining whether cancer has spread and, if so, how far. There are various staging systems that can be used for neuroblastoma. Always consult your child's physician for information on staging.
- Blood tests, such as a complete blood count, blood chemistries, kidney and liver function tests, and ferritin.
- Urine tests, such as urinalysis, Homovanillic.
- Computer-assisted tomography scan (CT or CAT scan) is a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
- Magnetic resonance imaging (MRI) is a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
- X-ray is a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
- Ultrasound (sonography) is a diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.
- Bone scans takes pictures or x-rays of the bone after a dye has been injected that is absorbed by bone tissue. These are used to detect tumors and bone abnormalities.
- MIBG (metaiodobenzylguanidine) scan uses MIBG and radioactive iodine, which is a highly sensitive and specific marker for neuroblastomas, to locate and diagnose tumors.
- Bone marrow aspiration and/or biopsy, which involves taking a small amount of bone marrow fluid (aspiration) and/or solid bone marrow tissue (called a core biopsy), usually from the hip bones, to be examined for the number, size, and maturity of blood cells and/or abnormal cells.
- Spinal tap/lumbar puncture. A special needle is placed into the lower back, into the spinal canal. This is the area around the spinal cord. The pressure in the spinal canal and brain can then be measured. A small amount of cerebral spinal fluid (CSF) can be removed and sent for testing to determine if there is an infection or other problems. CSF is the fluid that bathes your child's brain and spinal cord.
One method of staging neuroblastoma is the following:
Back to Top
- Stage 1 involves a tumor that does not cross the midline of the body, is completely resectable(surgically excised), and has not spread to other areas of the body. The lymph nodes on the same side of the body as the tumor do not have cancer cells present.
- Stage 2A involves a tumor that does not cross the midline of the body, but is not completely resectable. This stage of tumor has not spread to other areas of the body, and lymph nodes on the same side as the tumor do not have tumor cells present.
- Stage 2B involves a tumor that may or may not be completely resectable, has not spread to other areas of the body, but lymph nodes on the same side of the tumor have tumor cells present. Lymph nodes on the opposite side of the tumor must be negative for tumor cells in this stage of disease.
- Stage 3 involves a tumor that crosses the midline of the body, is not completely resectable, and lymph nodes are positive for tumor cells. This stage also includes a tumor that does not cross the midline, but the lymph nodes on the opposite side also contain tumor cells.
- Stage 4 involves a tumor that has metastasized to distant lymph nodes, bone marrow, liver, skin, and/or other organs (except as defined in stage 4S).
- Stage 4S involves a tumor that has metastasized (spread) to liver, skin, and/or bone marrow (includes minimal bone marrow involvement; more extensive bone marrow involvement should be classified as stage 4).
Specific treatment for neuroblastoma will be determined by your child's physician based on the best available treatment for child’s disease and consideration of the:
- Child's age, overall health, and medical history.
- Type, location, and size of the tumor.
- Extent of the disease.
Treatment may include (alone or in combination):
- Surgery for tumor and/or metastatic resection, and removal of lymph nodes involved.
- Radiation to shrink the remaining tumor or treat metastasis and/or recurrent disease.
- Blood and marrow transplant.
- Biologic therapy of retinoic acid and antibodies.
- Supportive care for the side effects of treatment.
- Antibiotics to prevent/treat infections.
- Continuous follow-up care to determine response to treatment, recurrent disease, and late effects of treatment.
Treatment options should be discussed with the child's physician.
Back to Top
Long-term outlook for a child with neuroblastoma
Prognosis greatly depends on the:
Other factors that may play role in the outcome include:
- Child’s age.
- Extent of the disease (presence or absence of metastasis).
- Biologic factors of the tumor, such as MYCN status.
As with any cancer, prognosis and long-term survival can vary greatly from individual to individual. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with neuroblastoma. Side effects of radiation, chemotherapy and surgery, as well as recurrence of the disease, can occur in survivors of neuroblastoma. New methods and developments in treatment approaches are continually being tested to improve the outcome of patients with neuroblastoma and to decrease side effects.
- Size and location of the tumor.
- Tumor’s response to therapy.
- Child’s tolerance of specific medications, procedures, or therapies.
Back to Top
Neuroblastoma - Departments & Programs - Children's National Medical Center