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 ganglion, the tumor may appear in the chest, neck, or paravertebral intra-abdominally. Polyhydramnios and fetal hydrops may accompany the lesion. The tumor can metastasize in utero (placenta, liver, blood vessels).