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Childhood hepatocellular carcinoma: a clinicopathological study of 12 cases with special reference to EpCAM
Yoh Zen1, Roshni Vara, Bernard Portmann
1Histopathology Section, Institute of Liver Studies, King's College Hospital, London, UK.
Insights
Childhood hepatocellular carcinoma (HCC) is characterized by diffuse epithelial cell adhesion molecule (EpCAM) expression, distinguishing it from adult HCC and other pediatric liver tumors. This finding may suggest immature neoplastic cells in pediatric HCC.
Area of Science:
- Pediatric oncology
- Hepatocellular carcinoma research
- Tumor marker analysis
Background:
- Hepatocellular carcinoma (HCC) is rare in children.
- Understanding pediatric HCC characteristics is crucial for diagnosis and treatment.
- Comparison with adult HCC, fibrolamellar HCC, and hepatoblastoma is needed.
Observation:
- Twelve pediatric HCC cases were analyzed and compared to adult HCC, fibrolamellar HCC, and hepatoblastoma.
- Childhood HCCs arose in the context of specific underlying conditions like tyrosinaemia type 1 and bile salt export transporter deficiency.
- Morphological and immunohistochemical features were evaluated.
Findings:
- Childhood HCC consistently showed diffuse epithelial cell adhesion molecule (EpCAM) expression, particularly in younger children.
- EpCAM expression was focal in adult HCC, differentiating it from pediatric cases.
- Other markers like CK7, β-catenin, and p53 also distinguished childhood HCC from fibrolamellar HCC and hepatoblastoma.
Implications:
- Diffuse EpCAM expression is a potential hallmark of pediatric HCC.
- This marker may indicate immature neoplastic cells in childhood liver cancer.
- Further research into EpCAM's role could improve diagnostic accuracy and therapeutic strategies for pediatric HCC.
Aims:
To elucidate the characteristics of hepatocellular carcinoma (HCC) in children.
Methods And Results:
A retrospective search of our database identified 12 children with HCC (aged 10 months to 11 years; male/female ratio of 5:7). Their pathological features were compared with those of adult HCCs (n = 20), fibrolamellar HCCs (n = 14), and hepatoblastomas (n = 15). All childhood HCCs developed on a background of cirrhosis resulting from tyrosinaemia type 1 (n = 4), bile salt export transporter deficiency (n = 4), biliary atresia (n = 3), and long-standing total parenteral nutrition (n = 1). HCCs in cases of tyrosinaemia type 1 always had clear cell changes, solid architecture, and only mild nuclear atypia, whereas the morphological features of HCCs in the other conditions were basically similar to those of adult HCCs. On immunostaining, all cases of childhood HCC were positive for epithelial cell adhesion molecule (EpCAM); expression was diffuse (>50% of cancer cells) in 11 cases, and particularly strong in six children, all aged <3 years. In contrast, EpCAM was only focally expressed in three cases of adult HCC (15%). EpCAM was also expressed in most fibrolamellar HCCs and hepatoblastomas, but these two neoplasms differed from childhood HCCs in the expression of CK7, β-catenin, and p53.
Conclusions:
The diffuse expression of EpCAM characterizes childhood HCC, and may indicate immaturity of neoplastic cells.
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