The role of liver transplantation in the management of paediatric liver tumours

Abstract

In recent years, considerable progress has been made in the treatment of children with hepatoblastoma largely due to effective pre-operative chemotherapy. Total hepatectomy and liver transplantation has emerged as an effective treatment for the small proportion of children with unresectable hepatoblastoma limited to the liver. A 5-year survival of 70% can be achieved in such cases. In contrast, the results of liver transplantation in children with hepatocellular cancer remain poor because these tumours are usually advanced with evidence of major vascular invasion and/or extrahepatic spread at the time of presentation. An exception is those children in whom the hepatocellular carcinoma is detected during surveillance of chronic liver disease - they typically have smaller tumours and frequently have a good prognosis after liver transplantation. The role of liver transplantation in children with other primary hepatic malignancies remains uncertain because experience is very limited. Liver transplantation is rarely needed in the management of children with benign liver tumours but, if other treatments have failed, it can be a life-saving intervention.

Figure 1

SIOPEL pretreatment (PRETEXT) grouping system for malignant liver tumours in children.

Figure 2

Unresectable multifocal PRETEXT IV hepatoblastoma in a 3-year-old child treated by liver transplantation. Axial magnetic resonance scan with gadolinium enhancement showing wide-spread multifocal tumour.

Figure 3

Coronal magnetic resonance scan of a large unresectable PRETEXT III hepatoblastoma in a 7-month-old infant, only partially responsive to chemotherapy. Treated by total hepatectomy and liver transplantation.

Figure 4

Unresectable hepatocellular carcinoma in a 13-year-old girl without underlying liver disease. PRETEXT IV tumour with extensive vascular invasion. The arrow demonstrates tumour in the main portal vein on this coronal magnetic resonance image.

Figure 5

Massive hepatic haemangioendothelioma in a 7-monthold infant treated by liver transplantation. (A) Clinical; (B) Axial CT scan after intravenous contrast; and (C) operative appearances. The child remains well 2 years later.

Figure 5

Massive hepatic haemangioendothelioma in a 7-monthold infant treated by liver transplantation. (A) Clinical; (B) Axial CT scan after intravenous contrast; and (C) operative appearances. The child remains well 2 years later.

Figure 5

Massive hepatic haemangioendothelioma in a 7-monthold infant treated by liver transplantation. (A) Clinical; (B) Axial CT scan after intravenous contrast; and (C) operative appearances. The child remains well 2 years later.

Figure 6

(A) Axial magnetic resonance image of a 7-year-old boy with an aggressive hilar inflammatory myofibroblastic tumour (arrows). (B) Gross appearance of resection specimen after hepatectomy and OLT. Note the dense hilar infiltration, left lobe atrophy and satellite nodules (arrows).

Figure 6

(A) Axial magnetic resonance image of a 7-year-old boy with an aggressive hilar inflammatory myofibroblastic tumour (arrows). (B) Gross appearance of resection specimen after hepatectomy and OLT. Note the dense hilar infiltration, left lobe atrophy and satellite nodules (arrows).