Abstract
Congenital hepatoblastoma is rare and defined as hepatoblastoma diagnosed before birth or within one month after birth. A male infant presented with hepatic tumor rupture immediately after vaginal delivery at 38 weeks of gestation. The serum alpha-fetoprotein (AFP) level on day 9 was 264,109 ng/ml, which was within the normal range, but the ratio of lectin-reactive alpha-fetoprotein (AFP-L3%) was high at 29%. Differential diagnosis from hepatic hemangioma was difficult, necessitating biopsy. Images disclosed a large tumor in the right lobe occluding the inferior vena cava. Four cycles of chemotherapy from day 29 recanalized the inferior vena cava, and the tumor could be removed by resection of the right lobe and caudate lobe. The AFP level decreased within the normal range soon after surgery, and AFP-L3% was not detected. He was discharged after an additional 2 cycles of chemotherapy. He is well without any evidence of recurrence 1 year and 4 months after surgery. Data collected from the literature showed that the AFP levels of congenital hepatoblastoma are within the normal range during the first half of the neonatal period. The presented case suggests that AFP-L3% may be useful for diagnosis and as a therapeutic effect determination marker. Despite previous reports of poor prognosis, congenital hepatoblastoma, as well as ordinary hepatoblastoma, can be treated by the combination of careful chemotherapy and surgery.
