Hepatoblastoma: the liver tumour of early childhood
- Lesion
Hepatoblastoma is the most common liver cancer of small children; it is made of cells that look like fetal liver.
What it is
Hepatoblastoma is the most common primary liver malignancy of children, usually before age three. It recapitulates the developing liver, with fetal and embryonal epithelial cells, sometimes mixed with mesenchymal elements such as osteoid. Almost all tumours activate the Wnt/β-catenin pathway. It is more frequent with familial adenomatous polyposis, Beckwith-Wiedemann syndrome and very low birth weight. AFP is usually very high and is used for diagnosis and follow-up.
Structure
- Gross: large, usually solitary, well-circumscribed tan mass with hemorrhage and necrosis.
- Micro: fetal cells resembling fetal hepatocytes in thin trabeculae with light and dark areas; embryonal cells in rosettes or tubules; extramedullary hematopoiesis; mixed type adds osteoid, cartilage or primitive mesenchyme.
Step by step
- A germline or somatic defect activates Wnt/β-catenin signalling in hepatic progenitor cells.
- β-catenin accumulates in nuclei and drives proliferation genes.
- Progenitors fail to mature and form a tumour resembling fetal and embryonal liver.
- The tumour grows as a large abdominal mass and secretes AFP.
Markers
- Serum alpha-fetoprotein
- Nuclear β-catenin
- Glypican-3
Clinical notes
- An asymptomatic abdominal mass found by a parent; sometimes precocious puberty from hCG secretion.
- Children with FAP or Beckwith-Wiedemann syndrome are screened in early childhood.
Test yourself
Think of the answer, then tap the question to see it.
What is the most common primary malignant liver tumour of young children?
Hepatoblastoma.
Which signalling pathway is activated in nearly all hepatoblastomas?
The Wnt/β-catenin pathway, through CTNNB1 mutations or APC loss.
Which syndromes predispose to hepatoblastoma?
Beckwith-Wiedemann syndrome and familial adenomatous polyposis; very low birth weight is also a risk factor.
Connections
Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.
Sources: Childhood Liver Cancer Treatment (PDQ®)–Health Professional Version (National Cancer Institute, 2026).
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Representative model, for educational purposes only.