Hepatoblastoma and liver tumours in children

 

Liver tumours are rare in children. They represent 1% of all solid paediatric tumours and are malignant in 2/3 of all cases. Two types of tumours account for 90% of malignant tumours (excluding metastases). First of all, the hepatoblastoma which appears every year in 1 in a million children and usually occurs before the age of three. Second, the hepatocellular carcinoma which is much rarer and more likely occurs in adolescents. The carcinoma may be associated with a pre-existing liver disease and, in this case, it manifests earlier in childhood. Finally, there is a third type of tumour, which, however, is much rarer than hepatoblastoma and hepatocellular carcinoma, this is the sarcoma. It represents 10% of all liver tumours and generally affects older children. Benign liver tumours are mainly of vascular origin (haemangioma, haemangioendothelioma), followed in order of frequency by mesenchymal hamartoma, focal nodular hyperplasia and adenoma.

The main symptoms of all these liver tumours are a generally palpable liver and abdominal pain. High-quality imaging including computer tomography (CT-Scan or scanner) or magnetic resonance imaging (MRI) is required for the diagnosis and for the preoperative assessment of the tumour size. In many cases, a biopsy of the tumour must be performed under general anaesthesia.

The treatment of malignant tumours aims at achieving complete surgical removal. This operation is nearly always combined with chemotherapy before and after the operation. When the tumour cannot be removed by surgery, liver transplant may be an option to cure the child. The progresses achieved in medical and surgical healthcare have contributed considerably to the improvement of the prognosis of children affected by one of these tumours.

Benign tumours, however, are monitored. When these tumours turn out to be locally invasive, they are treated either by ablation surgery or by procedures of interventional radiology.

Dr. Eliane Rahal

2011