Neuroblastoma is a cancerous tumor that begins in nerve tissue of infants and very young children. Neuroblastoma is rare in children older than 10-years-old. However, it does occasionally occur 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 one on top of each 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.
Most children affected by neuroblastoma have been diagnosed before 5-years-old. In rare cases, neuroblastoma can be detected before birth by a fetal ultrasound.
In the U.S., approximately 500 children are diagnosed with neuroblastoma each year. It is the most common tumor found in children younger 1-year-old. For unknown reasons, it occurs slightly more often in males than in females.
Neuroblastoma cancer cells can spread (metastasize) quickly to other areas of the body (e.g. lymph nodes, liver, lungs, bones, central nervous system and bone marrow). Approximately 70% of all children diagnosed with neuroblastoma will have some metastatic disease.
A variety of chromosome abnormalities may present in 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 MYCN. The amplification of MYCN causes uncontrolled cell growth. A variety of other chromosome abnormalities may also be present in neuroblastoma.
It is estimated that many neuroblastoma cases result from 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.
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 a patient is about 1%. 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.
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.
In addition to a complete medical and physical examination, diagnostic procedures for neuroblastoma may include the following:
Including a complete blood count, blood chemistries, kidney and liver function tests, and a urinalysis.
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.
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.
A diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs onto film.
A diagnostic imaging technique which 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.
Pictures or x-rays taken of the bone after a dye has been injected that is absorbed by bone tissue. These are used to detect tumors and bone abnormalities.
Similar to a bone scan except that a special dye is injected that may be absorbed by neuroblastoma tissue throughout the body.
A procedure that involves a small amount of bone marrow fluid and tissue to be taken, usually from part of the hip bones to look for the presence of tumor cells.
Diagnosing neuroblastoma also involves staging and classifying the disease which determines treatment options and prognosis. Staging is the process of determining whether a 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.
One method of staging neuroblastoma is the following:
Specific treatment for neuroblastoma will be determined by your child's physician based on:
Treatment may include (alone or in combination):
Treatment options should be discussed with your child's physician.
Prognosis greatly depends on:
As with any cancer, prognosis and long-term survival can vary greatly from child to child. Prompt medical attention and aggressive therapy are important for the best prognosis.