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Definition: This
tumor arises from undifferentiated neural tissue of the
adrenal medulla or sympathetic ganglia in the abdomen, thorax,
pelvis, or head and neck. Usually, the lesion is isolated, but
occasional metastasis before birth, may occur.
Prevalence: This is one of the most common tumors of infancy with an incidence
of 1 in 10,000 to 1 in 30,000 live births.
Etiology: The
etiology is unclear and most cases appear sporadic.
Ultrasound features: The sonographic picture is characterized by a
cystic, solid,
or complex mass in the region of the adrenal gland,
directly above the level of the kidney and under the diaphragm.
Occasionally, calcifications are apparent within the tumor. If
arising in a sympathetic ganglia, the tumor may appear in the
chest, neck, or paravertebral intra-abdominally. Polyhydramnion and fetal
hydrops may accompany the lesion. The tumor can metastasize in utero (placenta, liver, blood vessels).
Prognosis: The two most
important factors affecting the prognosis are the age
of the patient and the stage of the disease. Patients less than 1 year of age
have a more favorable prognosis. When
identified in-utero, neuroblastomata are associated with an excellent
outcome. The overall survival rate is only 10% to 20% in children older than 1
year of age, but greater than 90% in
patients younger than 1 year of age at the time of diagnosis (21).
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| fig. 10 |
| 21. | Grosfeld, J.A., Rescorla, F.J., West, K.W., Goldman, J. Neuroblastoma in the first year of life: clinical and biologic factors influencing outcome. Semin Pediatr Surg 1993; 2:37-43. |
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