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Key Points

  • Liver tumors account for only 5% of fetal and neonatal tumors.

  • The most common tumor is hemangioma followed by mesenchymal hamartoma and hepatoblastoma.

  • Hemangiomas can be hypoechoic, hyperechoic, or undetectable and can result in high-output failure polyhydramnios and hydrops.

  • Mesenchymal hamartomas tend to be cystic lesions.

  • Hepatoblastomas are typically solid and echogenic and occasionally have calcifications.

  • Hepatoblastoma can be associated with Beckwith–Wiedemann syndrome, familial adenomatous polyposis coli, and rarely, trisomy 18.

  • Alpha-fetoprotein levels may be markedly elevated in mesenchymal hamartoma and neuroblastoma.

  • In utero cyst decompression, rare mesenchymal hamartomas with extremely large cysts may be considered.

  • Transplacental steroids may be helpful in large hemangiomas associated with high-output cardiac failure or hydrops.

  • There is increased risk of intrauterine demise and stillbirth in primary hepatic tumors.

  • Neonatal management of primary hepatic tumor is complicated and should be performed at centers with appropriate expertise.


Tumors of the liver are rare during the perinatal period. They account for only 5% of all neoplasms that occur in the fetus and the newborn (Campbell et al., 1987; Borch et al., 1992; Broadbent, 1992; Werbe et al., 1992; Isaacs, 1997). The most common primary hepatic tumor is hemangioma, followed by mesenchymal hamartoma and hepatoblastoma (Stocker and Ishak, 1983; Isaacs, 1985; Davis et al., 1988; Davenport et al., 1995; von Schweinitz, 2003; Laberge et al., 2005; Christison-Lagay et al., 2007; Isaacs, 2007). Each has been detected prenatally by ultrasound examination and can be detected postnatally by palpation of an abdominal mass (Romero et al., 1988; Garmel et al., 1994; Isaacs, 1997; Isaacs, 2007). However, metastatic lesions are more common than primary liver tumors (Dehner, 1978; Coffin and Dehner, 1992). The most common tumor that metastasizes to the liver in the fetus and newborn is neuroblastoma, followed by leukemia, yolk sac tumor from sacrococcygeal teratoma, and rhabdoid tumor of the kidney (Dehner, 1978; Isaacs, 1985, Isaacs, 1997). The majority of hepatic hemangiomas are diagnosed before the age of 6 months and almost 50% appear within the first week of life (Dehner et al., 1975; Laird et al., 1976; Stanley et al., 1977, Dehner, 1978, Dehner, 1981, Ehren et al., 1983; Miller and Greenspan, 1985a and Miller and Greenspan, 1985b; Golitz et al., 1986; Dehner, 1987; Davis et al., 1988; Luks et al., 1991; Drut et al., 1992; Werbe et al., 1992). There have been multiple reports in the literature of hepatic hemangiomas diagnosed by prenatal sonographic examination (Nakamoto et al., 1983; Platt et al., 1983; Horgan et al., 1984; Petrovic et al., 1992; Sepulveda et al., 1993; Chou et al., 2005; Isaacs, 2007). In the report by Isaacs, reviewing reported cases in the literature and experience at Children’s ...

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