A rare tumour of childhood, but the most common liver tumour of childhood (ie. liver tumours are very very rare in childhood).
Epidemiology
Most occur by the age of 5 years, mostly in males.
Aetiology
The cause is unknown, but increased frequency is seen in many syndromal conditions such as Beckwith-Wiedemann, trisomy 21, and familial adenomatous polyposis syndrome.
Natural History
Most cases are resectable with pre-operative chemotherapy and the availability of liver transplantation.
Clinical Features
Symptoms and Signs
Most children are found to have a painless abdominal mass. They may suffer from weight loss.
Bloods
AFP is elevated (similar to hepatocellular carcinoma) in most cases. Anaemic and thrombocytosis are common.
Imaging
CT is the best imaging modality, and lesions may be calcified.
Tumour Features
Macroscopy
Tumours are large, of variable colour, and may show areas of haemorrhage or necrosis. THey are usually encapsulated.
Microscopy
A variety of patterns is seen:
- Fetal epithelial differentiation (trabeculated, sinusoids)
- Combined fetal/embryonal (as above but with a population of small round blue cells)
- Small cell undifferentiated (small round blue cell tumour)
- Mixed epithelial and mesenchymal (as for combined tumour but with added mesenchymal elements)
Genetics
Trisomy of chromosomes 2 or 20 is frequent.
