- 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%).
- 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.
- No increased risk of chromosomal abnormalities or genetic syndromes.
- Detailed ultrasound examination.
- MRI to detect possible intraspinal extension of the tumor.
- Ultrasound scans every 4 weeks to monitor the size of the tumor and development of development of hepatomegaly, placentomegaly, heart failure and polyhydramnios.
- Place: hospital with neonatal intensive care.
- Time: 38 weeks.
- Method: induction of labor aiming for vaginal delivery.
- 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.
- No increased risk of recurrence.