- 1 in 100,000 births.
- The most common are hemangioma, mesenchymal hamartoma and hepatoblastoma.
- They can be cystic, echogenic, mixed solid and cystic or they may just present with hepatomegaly.
- Hemangiomas and hepatoblastomas may be associated with evidence of high-output heart failure, hydrops and polyhydramnios.
- Color Doppler studies may be helpful in distinguishing hemangioma from the other tumors.
- Chromosomal abnormalities: no increased risk.
- Hepatoblastoma can be associated with the Beckwith–Wiedemann syndrome with evidence of organomegaly and macroglossia.
- Detailed ultrasound examination, including echocardiography.
- Doppler ultrasound to define the vascular anatomy of the liver.
- Fetal MRI to define the extent of the tumor and its relationship with intrahepatic structures and adjacent organs.
- Ultrasound scans every 2-3 weeks to monitor the size of the tumor and development of heart failure and polyhydramnios.
- In the case of large hemangiomas associated with high-output heart failure maternal administration of steroids may arrest the growth of the tumor.
- Place: hospital with neonatal intensive care and pediatric surgery.
- Time: 38 weeks.
- Method: cesarean section for large vascular tumors to avoid rupture at the time of delivery.
- Hemangiomas: survival of about 80% after treatment with steroids. However, occasionally, they are associated with arteriovenous shunting, congestive heart failure and hydrops, resulting in intrauterine or neonatal death. After infancy the tumors regress spontaneously.
- Mesenchymal hamartoma: surgical resection with 70% survival.
- Hepatoblastoma: surgical resection with 60% survival. Prior to resection several cycles of chemotherapy may be necessary to shrink the tumor and make it resectable.
- In general there is no increased risk of recurrence.
- In hepatoblastoma in association with Beckwith–Wiedemann syndrome (autosomal dominant) and familial adenomatous polyposis (autosomal dominant condition that predisposes to colonic adenomas and carcinomas) the risk is increased.