Neuroblastoma
Neuroblastomasare the most common abdominal tumor in childhood. The
tumors arise from greatly undifferentiated and invasive cells from the sympa-
thetic nervous system. Tumors usually occur in the abdomen near the adrenal
gland or spinal ganglia but may also be noted in the head, neck, chest, or
pelvis. Neuroblastomas occur primarily in infants <2 years of age (50%) and
preschool children (25%) and slightly more often in males than females.
Metastasis is most commonly to the bone marrow, liver, and subcutaneous
tissue.
Signs and Symptoms
Common assessment findings:
- Symptoms depend on the location and stage of the tumor (secondary
to pressure on adjacent structures—respiratory distress from thoracic
mass). - Cervical or supraclavicular lymphadenopathy may be noted.
- Presents as a firm, irregular, painless mass in the abdomen that
crosses the midline. - Urinary retention or frequency may be noted if kidney is compressed.
- Periorbital edema, exophthalmos, and supraorbital ecchymosis are
noted with metastasis to the retrobulbar tissue. - Weight loss and anorexia may be noted due to tumor pressure.
- Difficulty swallowing and edema of the neck and face may occur
from compression on the vena cava. - Jaundice from liver pressure may be noted and if metastasis to the skin
has occurred, blue or purplish nodules may be noted on the arms or legs. - Flushed face, excessive sweating, and hypertension may result from
adrenal pressure. - Symptoms of neurologic impairment, including paralysis from com-
pressed nerves, may be noted, as well as neurologic changes from
intracranial lesions.
Test Results
Diagnosis by biopsy.
Skeletal survey of skull, chest, abdomen, and bone marrow aspirates.
Blood count, urinalysis (may reveal catecholamine excretion from
adrenals).
CT scan and bone scans to detect metastasis.
Vanillylmandelic acid (VMA), homovanillic acid (HVA), dopamine in
urine (breakdown products of catecholamines) may be noted with an
adrenal tumor.
Increased ferritin, neuron-specific enolase (NSE), and ganglioside(GD2)
may be noted (isoenzymes from neurons).
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