Prevalence:

• 1 in 20,000 births.
• Arises from undifferentiated neural tissue of the adrenal medulla (90%) or sympathetic ganglia in the abdomen, thorax, pelvis, or head and neck (10%).

Ultrasound diagnosis:

• Cystic, solid, or complex mass in the region of the adrenal gland, usually in the third trimester. Occasionally, calcifications are present.
• Tumors arising from the sympathetic ganglia may appear in the abdomen, thorax or neck.
• The tumor can metastasize in utero to the placenta, umbilical cord or liver.
• There may be associated polyhydramnios and fetal hydrops.

Associated abnormalities:

• No increased risk of chromosomal abnormalities or genetic syndromes.

Investigations:

• Detailed ultrasound examination.
• MRI to detect possible intraspinal extension of the tumor.

Follow up:

• Ultrasound scans every 4 weeks to monitor the size of the tumor and development of development of hepatomegaly, placentomegaly, heart failure and polyhydramnios.

Delivery:

• Place: hospital with neonatal intensive care.
• Time: 38 weeks.
• Method: induction of labor aiming for vaginal delivery.

Prognosis:

• Survival is >90% if the diagnosis is made in utero or in the first year of life, but <20% for those diagnosed after the first year. Treatment requires surgical excision of the tumor and chemotherapy.

Recurrence:

• No increased risk of recurrence.