Liver Tumours ✅ Flashcards
Give 2 liver tumours seen in childhood
- Hepatoblastoma
- Hepatocellular carcinoma
What is the most common type of liver tumour seen in childhood?
Hepatoblastoma
What age does hepatoblastoma present in?
<4 years
What age is hepatoblastoma most commonly seen in?
<18 months
What are the common presenting features of hepatoblastoma?
- Palpable abdominal mass with abdominal distention
- Anorexia
- Weight loss
- Vomiting
- Jaundice
What should physical examination focus on in suspected hepatoblastoma?
Associated syndromes
What syndromes are associated with hepatoblastoma?
- Beckwith-Wiedemann syndrome
- Familial adenomatous polyposis
- Glycogen storage disease type I
Why is a detailed family history important in suspected hepatoblastoma?
To identify possible risk factors, e.g. bowel cancer at a young age (suggesting FAP)
What is typically found on blood tests in hepatoblastoma?
Raised alpha-fetoprotein
What is the clinical use of alpha-fetoprotein in hepatoblastoma?
- Diagnosis
- Monitoring response to treatment
What is AFP?
A plasma protein which represents the fetal form of albumin
By what age does AFP fall to undetectable levels?
By 8 months of life
What does a positive AFP indicate after 8 months?
A pathology, most likely a tumour
What kinds of tumours can cause a raised AFP?
- Hepatic
- Yolk sac
What produces AFP?
The yolk sac and liver (and when yolk sac involuted, liver alone)
How is hepatoblastoma treated?
Chemotherapy followed by resection or transplantation
What chemotherapy agent is used in hepatoblastoma?
Cisplatin
What is the treatment plan for hepatoblastoma dependent on?
- Extent of disease
- Residual metastases following treatment
What can hepatocellular carcinoma occur secondarily to?
- Underlying progressive chronic liver disease and cirrhosis
- Metabolic disease
- Infective causes
Give 3 metabolic causes of hepatocellular carcinoma?
- Ornithine transcarbamylase (OTC) deficiency
- Tyrosinaemia
- Progressive familial intrahepatic cholestasis (PFIC)
Give 2 infective causes of hepatocellular carcinoma?
- Hepatitis B
- Hepatitis C
How is hepatocellular carcinoma managed?
Resection, often in combination with adjuvant chemotherapy or liver transplantation
When is liver transplantation an option in hepatocellular carcinoma?
In the absence of distant metastases
Why is the risk of hepatocellular carcinoma higher in chronic liver disease?
Any chronic liver disease and cirrhosis has the potential for dysplastic change
What should be done due to the higher risk of hepatocellular carcinoma in chronic liver disease?
All chronic liver disease should be monitored with regular ultrasound examinations of the liver and AFP
