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Hepatocellular carcinoma in biliary atresia: King's College Hospital experience
Nedim Hadžić1, Alberto Quaglia, Bernard Portmann
1Paediatric Liver Centre, King's College Hospital, Denmark Hill, London, United Kingdom. nedim.hadzic@kcl.ac.uk
Insights
Hepatocellular carcinoma (HCC) risk is low in children with biliary atresia (BA), a common childhood liver disease. Liver transplantation is an effective treatment for HCC in these patients.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Oncology
Background:
- Biliary atresia (BA) is the most common chronic liver disease in childhood.
- Hepatocellular carcinoma (HCC) is a rare but serious complication in children with BA.
Purpose of the Study:
- To determine the risk of developing HCC in pediatric patients diagnosed with BA.
- To analyze the clinical, demographic, and histological features of HCC in children with BA.
Main Methods:
- Retrospective analysis of a tertiary referral center database.
- Identification of children with BA who developed or were incidentally found to have HCC.
- Review of demographic, clinical, radiological, and histological data.
Main Results:
- Three of 387 infants (0.8%) diagnosed with BA developed HCC post-operation.
- Two additional patients were diagnosed with HCC on liver explants.
- All five children with HCC underwent successful liver transplantation.
Conclusions:
- HCC develops in a small percentage of children with BA.
- Alpha-fetoprotein levels and ultrasound screening aid in detection but are not definitive.
- Liver transplantation is an effective treatment for HCC in BA patients without extrahepatic involvement.
Objectives:
To establish risks for development of hepatocellular carcinoma (HCC) in children with biliary atresia (BA), the most common chronic liver disease of childhood.
Study Design:
In our tertiary referral center database we have identified children with BA who had development of or have been incidentally found to have HCC. Their demographic, clinical, radiologic, and histologic features were analyzed.
Results:
Between 1990 and 2008, 387 infants were diagnosed with BA at our center. Of these, three (0.8 %) who underwent operation at a median age of 68 (range 66 to 71) days had development of a histologically proven HCC detected at a median age of 2.1 (range 1.8 to 4.9) years. Another two, referred later, were diagnosed with HCC on their liver explants at ages 1.1 and 17.75 years, respectively. Overall, two had elevated serum levels of alpha-fetoprotein. All five children underwent successful liver transplantation at a median age of 2.1 years (range 1.1 to 17.75) and remain well after a median of 2.5 (range 2 to 5.7) years.
Conclusion:
HCC develops in a small percentage of children with BA. Serum alpha-fetoprotein levels and ultrasound screening are helpful but not absolute markers of the malignant change. In the absence of the extrahepatic involvement, liver transplantation represents an effective treatment.