CHILD NEUROBLASTOMA
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ALTERNATE NAMES
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Congenital Neuroblastoma; Sympathicoblastoma; Stage IVS Neuroblastoma; Pepper’s syndrome;
Schwannian Stroma-Poor Neuroblastoma
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DESCRIPTION
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Neuroblastoma is a malignant solid tumor that develops from the nerve tissues that
form the sympathetic nervous system (the part of the nervous system that controls
body functions, such as heart rate, blood pressure, digestion, and levels of certain
hormones). The cause of neuroblastoma is unknown. Most neuroblastomas begin in the
abdomen near the adrenal glands, or next to the spinal cord, or in the chest. Child Neuroblastoma with distant metastases or recurrent is considered an aggressive tumor because it often quickly spreads to other parts
of the body.
The first symptoms are usually fever, a general sick feeling (malaise), and pain.
There may also be loss of appetite, weight loss, and diarrhea. More specific signs
and symptoms depend on the type of neuroblastoma.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: Diagnostic tests include:
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Urine catecholamine levels (vanillylmandellic acid and homovanillic acid levels);
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Imaging tests (x-ray, ultrasound, CT scan, MRI, radionuclide bone scan);
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Physical findings: Presentation signs vary depending on the site of the tumor, such as:
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High blood pressure and a fast heart rate (caused by adrenal gland tumors); and
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ICD-9:
194.0
ICD-10: C74.9
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PROGRESSION
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Neuroblastoma occurs in infants and young children, and is frequently diagnosed before
age 5. It may occur in older children but only rarely.
Stage IV is considered advanced if the tumor has spread to other parts of the body,
most commonly the skin, liver or bone marrow. Stage IVS applies only to children younger
than 1 year old. However, despite the extent of metastasis, babies with Stage IVS
have a good chance of recovery, and sometimes the condition sometimes resolves without
any treatment.
Patients older than 18 months usually present with metastatic disease and have poor
outcomes despite intensive therapy. Most recurrences occur during the first 2 years
following treatment.
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TREATMENT
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Treatment varies depending on location of the tumor, the stage of the cancer, and
patient’s age. The types of treatment may include surgery, chemotherapy, and radiation
therapy. Stem cell transplantation may be needed for patients with high-risk disease.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Suggested Listings for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Pathology/biopsy report of the cancer; and
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Treatment records including surgical procedures, and up-to-date progress notes.
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DETERMINATION
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LISTING
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REMARKS
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Meets
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113.21 B
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Requires documentation of metastasis or recurrence.
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113.21 C
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Requires documentation of metastasis or recurrence.
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Equals
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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