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What is Neuroblastoma?

written by: Blaise Wellesley•edited by: lrohner•updated: 9/30/2010

Find out the causes, symptoms, and treatment options of the common childhood cancer, neuroblastoma.

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    Neuroblastoma is a cancerous tumor that develops in nerve tissues. It is the most common cancer in infants, and accounts for 7.8 percent of childhood cancers in the United States. Masses from this disease can form in the pelvis, neck, chest or abdomen.

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    Early symptoms of neuroblastoma can include fevers, pain, weight loss, and lack of appetite. The locations of the tumor may vary. Other signs include:

    • Inability to fully empty the bladder
    • Paralysis of the legs or feet
    • Uncontrolled eye, leg, or feet movements
    • Flushed skin
    • Sweating
    • Rapid or irregular pulse

    Location specific symptoms can include:

    • Abdomen – swollen or engorged stomach
    • Bones – tenderness and pain in the bones
    • Chest – may have difficulty in breathing
    • Spinal cord – cause weakness and lack of balance

    Neuroblastoma can spread to other parts of the body, and 50 to 60 percent or more of cases metastasize to other organs. More commonly, neuroblastoma can occur the adrenal glands which sit above the kidneys.

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    Due to the generally early onset of neuroblastoma, research is being conducted around parental factors before and during pregnancy. As the cause is not well understood, a mutation in the gene ALK, anaplastic lymphoma kinase, has been seen in inherited neuroblastoma. ALK is key in the development of the brain and influences the central nervous system. Mutations in this gene allow cells to grow at an uncontrollable and rapid rate, ultimaterly causing neuroblastoma.

    Besides the possibility of this genetic component, other factors, such as exposure to chemicals, smoking and alcohol consumption, have been researched during pregnancy, and all results have been questionable. Other studies are researching the effects of infections in the womb, and the use of pregnancy drugs and hair dye.

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    A diagnosis can occur after a physical exams and tests when a doctor specializing in diseases, a pathologist, may confirm the diagnosis. These tests might include:

    • Full physical examination of the body: Used to check the body of general signs of health, lumps, or any other abnormalities characteristic of neuroblastoma.
    • Family history: If neuroblastoma is prevalent throughout the family, the doctor might want to investigate the family history of the patient in question. This could certainly rule out any genetic components the disease may have.
    • Urine test: Used to find elevated levels of metabolites in urine or the bloodstream. Since 90% of neuroblastoma cases have heightened levels, this test is very effective in diagnosis.
    • Blood test: Procedure that analyzes a blood sample to measure the amount of substances in the blood stream. These substances are released into the stream by organs from the body. Any decreased or increased values can show diseases in an organ. Example substances may be dopamine and norepinephrine which can be sign of neuroblastoma.
    • Cytogenetics: Investigates at the molecular level of the disease by directly analyzing any chromosomal abnormalities in cells.
    • Radioactive imaging: Using harmless radioactive particles, doctors can trace the pathway of certain molecules and find tumors in 90-95% of all patients with neruoblastoma. This procedure is a great way to monitor the disease after diagnosis.
    • Non-radioactive imaging: With the simple use of X-rays, CAT scans, and ultrasound exams, pictures are made of infected organs or tissues.
    • Microscopy/Biopsy: With a given tissue sample, pathologists are able to find certain patterns to help with diagnosis. The tissue is stained via chemicals and allows doctors to differential healthy and malignant cells in a sample.

    Given such a variety of tests available for diagnosing neuroblastoma, physicians may use one or many to help with the diagnosis. Also, if a test result is positive, another test might be issued to eliminate any chance of a false positive.

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    Treatment Options

    Just like any cancer, there are many options available depending on the severity of the tumor, the size and its ability to metastasize.

    Low-Risk and intermediate-risk neuroblastoma treatment options are:

    • Surgery in attempt to remove the tumor.
    • Waiting and watching if the tumor gets progressive worse.
    • Low-dose chemotherapy, when the tumor is already removed, or when surgery is ineffective.
    • Radiation therapy to eliminate tumors that do not disappear after chemotherapy.

    High-Risk treatments include:

    • High dose chemotherapy to decrease the size of the mass followed by surgery to remove as much as possible.
    • Radiation to the tumor and other parts of the body
    • Stem cell transplant, which can include bone marrow filtration and injection of new, non-cancerous stem cells.
    • New clinical trials to help with the disease. These treatments are new and not currently used in practice, however may help your child to find a cure. Can include antibody therapy, gene therapy, and even immunological therapies.
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    Even with the high number of treatment options available, relapse after treatment is common. Generally, 20 to 50 percent of high-risk neuroblastoma are unaffected by high-dose chemotherapy. Long-term survivors usually have complications due to treatment, and roughly two to three survivors will have an adverse influence from the treatment, and may lead to chronic issues 20 years after the initial diagnosis. As with any disease, they earlier the disease is caught and treatment started, the better off the child.

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