BACKGROUND/PURPOSE: Although hepatic tumors are uncommon in the perinatal period 
they are associated with significant morbidity and mortality in affected 
patients. The purpose of this review is to focus on the fetus and neonate in an 
attempt to determine the various ways liver tumors differ clinically and 
pathologically from those found in the older child and adult and to show that 
certain types of tumors have a better prognosis than others.
METHODS: The author conducted a retrospective review of perinatal hepatic tumors 
reported in the literature and of patients treated and followed up at Children's 
Hospital San Diego and Children's Hospital Los Angeles. Only fetuses and infants 
younger than 2 months with adequate clinical and pathologic data ere accepted 
for review. The period of patient accrual was from 1970 to 2005. Length of 
follow-up varied from 1 week to more than 5 years. Elevated alpha-fetoprotein 
level was defined as one significantly higher than that of the reporting 
institution's normal level for age group; laboratory values for this protein 
vary from one institution to the next and therefore it was not possible to 
assign one figure as a standard reference number. Discussion of the differential 
diagnosis and pathologic findings of hepatic tumors in the fetus and neonate are 
described elsewhere and will not be discussed here in detail (Perspect Pediatr 
Pathol 1978;4:217; Weinberg AG, Finegold MJ. Primary hepatic tumors in 
childhood. In: Finegold M, editor. Pathology of neoplasia in children and 
adolescents. Philadelphia, PA: WB Saunders, 1986; Am J Surg Pathol 1982;6:693; 
Pediatr Pathol 1983;1:245; Arch Surg 1990;125:598; Semin Neonatol 2003;8:403; 
Pediatr Pathol 1985;3:165; Isaacs H Jr. Liver tumors. In: Isaacs H Jr, editor. 
Tumors of the fetus and newborn. Philadelphia, PA: WB Saunders, 1997; Isaacs H 
Jr. Liver tumors. In: Isaacs H Jr, editor. Tumors of the fetus and infant: an 
atlas. Philadelphia, PA: WB Saunders, 2002).
RESULTS: One hundred ninety-four fetuses and neonates presented with hepatic 
tumors diagnosed prenatally (n = 56) and in the neonatal period (n = 138). The 
study consisted of 3 main tumors: hemangioma (117 cases, 60.3%), mesenchymal 
hamartoma (45 cases, 23.2%), and hepatoblastoma (32 cases, 16.5%). The most 
common initial finding was a mass found either by antenatal sonography or by 
physical examination during the neonatal period. Overall, hydramnios was next 
followed by fetal hydrops, respiratory distress, and congestive heart failure, 
which were often related to the cause of death. Half of the fetuses and neonates 
with hepatoblastoma had abnormally elevated serum alpha-fetoprotein levels 
compared with 16 (14%) of 117 of those with hemangioma and 1 neonate with 
mesenchymal hamartoma. There were 76 (65%) examples of solitary (unifocal) 
hemangiomas and 41 (35%) of multifocal (which included the entity diffuse 
hemangiomatosis) with 86% and 71% survival rates, respectively. Of 45 patients 
with mesenchymal hamartoma, of the 29 (64%) who had surgical resections, 23 
(79%) survived. Patients with hepatoblastoma had the worst outcome of the group, 
for only 8 (25%) of 32 were alive. Half of patients with either stage 1 or 3 
hepatoblastoma died; no patient with stage 4 survived. There was some 
relationship between histologic type and prognosis. For example, half of the 
patients with the pure fetal hepatoblastoma histology survived compared with 
those with fetal and embryonal histology where 30% survived. Fifteen of 32 
hepatoblastoma patients received surgical resection with or without 
chemotherapy, resulting in 7 (47%) of 15 cures. The 56 fetuses and 138 neonates 
with hepatic tumors (hemangioma, mesenchymal hamartoma, and hepatoblastoma) had 
survival rates of 75%, 64%, and 25%, respectively. The overall survival of the 
entire group consisting of 194 tumors was 125 or 64%.
CONCLUSIONS: The study shows that clinical findings in fetuses and neonates with 
hepatic tumors are less well defined than in older children. Survival rates are 
much lower as well. When the clinical course is complicated by associated 
conditions such as stillbirth, fetal hydrops, congestive heart failure, severe 
anemia, or thrombocytopenia, the mortality rate is much greater. If the patient 
is mature enough and in a clinical condition where he or she can be operated on, 
survival figures approach those of the older child. Some hepatic tumors have a 
better prognosis than others. Neonates with focal (solitary) hepatic hemangiomas 
have the best outcome and fetuses with hepatoblastoma the worst. Although 
infantile hemangioma undergoes spontaneous regression, it may be life 
threatening when congestive heart failure and/or consumptive coagulopathy occur. 
Mesenchymal hamartoma is a benign lesion best treated by surgical resection, 
which usually results in cure. However, there are fatal complications associated 
with this tumor, ie, fetal hydrops, respiratory distress, and circulatory 
problems owing to a large space occupying abdominal lesion and sometimes 
stillbirth, all contributing to the death rate. Hepatoblastoma, the major 
malignancy of the fetus and neonate, is treated primarily by surgical resection. 
Pre- or postoperative chemotherapy is reserved for those patients with 
unresectable tumors or metastatic disease. The survival rate is much lower than 
that reported by multigroup prospective trials. Patients die from the mass 
effect caused by the tumor, which lead to abdominal distension, vascular 
compromise, anemia, hydrops, respiratory distress, and stillbirth. Metastases to 
the abdominal cavity, lungs, and placenta are other causes of death. Because of 
the danger of labor-induced rupture of the tumor and potentially fatal 
intraabdominal hemorrhage, cesarean delivery is recommended when a hepatic tumor 
is found on prenatal ultrasound.