Rare hepatic malignancies
Intra-hepatic cholangiocarcinoma
[edit | edit source]- See separate topic
Hepatoblastoma
[edit | edit source]- Most common primary hepatic tumour of childhood (almost all cases occur before 3yo)
- Associated with FAP
- Derived from fetal or embryonic hepatocytic progenitors, and mesenchymal elements are often present
- Generally asymptomatic mass
- Most have elevated AFP
- Survival dependent on complete resection
- Long-term survival of 60-70% with complete resection
- Can cause pulmonary mets, which can also be excised
Sarcoma (metastatic lesions until proven otherwise)
[edit | edit source]- Angiosarcoma
- Associated with vinyl chloride or Thorotrast exposure
- Multiple hepatic masses, can appear in childhood
- Long-term survival uncommon
- Leiomyosarcoma
- Malignant fibrous histiocytoma
- Embryonic sarcoma
- Paediatric
- Primary hepatic rhabdoid tumours
- Paediatric
Non-Hodgkin lymphoma
[edit | edit source]- Can be primary liver lymphoma or secondary
- Treat the same as lymphoma elsewhere in the body
Primary hepatic neuroendocrine tumours
[edit | edit source]- Extremely rare
- Hard to distinguish from metastatic
- Liver is most common site for metastases
Malignant germ cell tumours (very rare - paediatric)
[edit | edit source]- Teratomas
- Choriocarcinomas
- Yolk sac tumours
Epithelioid haemangioendothelioma
[edit | edit source]- Rare
- Multiple bilateral hepatic masses
- Unpredictable clinical behaviour
- Transplantation has been successfully used to treat