Elsevier

Early Human Development

Volume 86, Issue 10, October 2010, Pages 637-642
Early Human Development

Fetal and neonatal liver tumours

https://doi.org/10.1016/j.earlhumdev.2010.08.023Get rights and content

Abstract

Liver tumours are rare in children and account for about 5% of all tumours in the fetus and newborn. The most frequently occurring are benign vascular tumours and mesenchymal hamartomas although malignancy in the form of hepatoblastoma is a possibility. While the diagnosis can be suspected antenatally (by ultrasound and MR scan), a precise diagnosis is often difficult due to the complexity of the tumours. Inutero development of such tumours may be associated with polyhydramnios, fetal hydrops and extreme cases the maternal mirror syndrome. Postnatal symptoms may include abdominal distension, cardiac failure, consumptive coagulopathy and bleeding due to tumour rupture, but is dependent on the nature of the actual tumour. Treatment options may include watchful waiting, surgical resection, hepatic artery embolisation/ligation and chemotherapy.

Introduction

Liver tumours presenting during the fetal or neonatal period are exceptional but with the advent of ubiquitous fetal ultrasonography are likely to become less of a rarity and more of a practical clinical problem.

There are no specific studies in this area to gauge a precise incidence, however 26/302 (8.6%) of paediatric liver tumours in a German national liver database (1989–2002) occurred in infants (< 6 weeks old) and of these over half were tumours of vascular origin [1]. Furthermore, in a recent review of the literature, Issacs et al. [2] highlighted the three likeliest candidate tumours. These are:

  • •

    Haemangioendothelioma/haemangioma (60%)

  • •

    Mesenchymal hamartoma (23%)

  • •

    Hepatoblastoma (16%)

We will review these in detail concentrating on clinical presentation and therapy, and conclude with some miscellaneous but quite definitely rare possibilities to complete the picture.

Section snippets

Embryology

The first recognisable embryonic precursors of liver may be identifiable from day 25 with an endodermal hepatic diverticulum developing from the foregut and expanding into the mesodermal stroma of the septum transversum. There is progressive expansion of vascular channels around cords of hepatoblasts. These are bipotential cells and precursors of biliary epithelial cells/cholangiocytes and mature hepatocytes. Notch signal activation (via Jagged1 receptor) appears to co-ordinate those fated as

Hepatic haemangioma/haemangioendothelioma

The pathogenesis of these “benign” vascular lesions is not clear and has led to some confusion in terminology. This is particularly true when trying to apply observations made in haemangioma/vascular malformation biology in general to those in the liver where there is usually a paucity of actual histological material. Thus, there appears to be true proliferative haemangioma with involution being evident after about 1 year — a characteristic type being the juvenile cutaneous haemangioma. These

Mesenchymal hamartoma

Mesenchymal hamartomas (MH) are typically heterogenous tumours comprised of cystic and solid elements and were first described only relatively recently in the 1950s. There is a slight male predominance and approximately 75% occur in the right lobe of the liver. Histologically they are lined by endothelium or bile duct epithelium. Sometimes one of the cysts may appear as the dominant feature but they do not communicate with the biliary tree and do not contain bile. The intervening stroma has a

Hepatoblastoma

Hepatoblastoma (HB) is the commonest malignant liver tumour in childhood, presenting typically in 2–3 year olds; < 10% are said to occur in the neonatal period [1], [2], [21]. There is a predominance in boys (2:1) and an increased risk in low birth weight babies. Although HB has a number of associations (e.g. familial adenomatous polyposis); only that with Beckwith–Weidemann has been reported in the neonatal period.

Miscellaneous liver tumours

There is a list to complement the above “common” diagnoses which are rare but still possible. Among the primary liver tumours described are germ cell tumours (including teratomas, yolk sac tumours and choriocarcinomas). Stage 4S neuroblastoma is a peculiar metastatic disease chiefly involving the liver which presents during the first two months of life. These might be mistaken for the multiple lesions of HAE. Perhaps surprisingly though, Stage 4S neuroblastoma has a good biological prognosis

Conclusion

Neonatal liver tumours are rare and accurate diagnosis can be challenging. In the UK, most are detected antenatally, but don't cause a clinical problem. In most, accurate diagnosis can await birth and a combination of CT or MR scans. There is a whole range of management options but active treatment is probably best organised in specialist centres.

Key guidelines

  • •

    Antenatal diagnosis with US and fetal MR scanning.

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    Actual antenatal intervention is usually unnecessary and should be used with caution.

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    Malignant transformation of mesenchymal hamartoma of the liver: case report and review of the literature

    J Pediatr Surg

    (1999)
  • P.E. North et al.

    A unique microvascular phenotype shared by juvenile hemangiomas and human placenta

    Arch Dermatol

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