[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [General pathology](https://durgical.com/en/general-pathology/) › [Liver and gallbladder](https://durgical.com/en/general-pathology/areas/liver/) › Hepatoblastoma: the liver tumour of early childhood

# 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.

Updated Nov 12, 2026

## Key points

- Most common pediatric primary liver cancer; peak in the first three years.
- Wnt pathway activation: CTNNB1 mutations or APC loss (FAP).
- Associations: Beckwith-Wiedemann syndrome (11p15), FAP, prematurity and very low birth weight.
- Epithelial type (fetal or embryonal) or mixed epithelial and mesenchymal type.
- Serum AFP markedly raised in most; low AFP is a poor sign.
- Treatment with chemotherapy and surgery (or transplant) cures most children.

## 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.

## Memory aid

A baby liver that never grew up: fetal and embryonal cells, β-catenin turned on, AFP sky high.

## 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.

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## Connections

## Related 3

- [Hepatocellular carcinoma](https://durgical.com/en/general-pathology/hepatocellular-carcinoma/)
- [Tumours of infancy and childhood](https://durgical.com/en/general-pathology/tumours-of-infancy-and-childhood/)
- [Tumour markers: serum and tissue, and what each one is for](https://durgical.com/en/general-pathology/tumour-markers-serum-and-tissue-and-what-each-one-is-for/)

## Related conditions 3

- [Beckwith–Wiedemann syndrome](https://durgical.com/en/conditions/beckwith-wiedemann-syndrome/)
- [Colorectal polyps and hereditary polyposis syndromes (FAP, Lynch)](https://durgical.com/en/conditions/colorectal-polyps-and-hereditary-polyposis-syndromes/)
- [Prematurity and its complications](https://durgical.com/en/conditions/prematurity-and-its-complications/)

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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](https://www.cancer.gov/types/liver/hp/child-liver-treatment-pdq) (National Cancer Institute, 2026).

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Representative model, for educational purposes only.

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Source: https://durgical.com/en/general-pathology/hepatoblastoma-the-liver-tumour-of-early-childhood/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/general-pathology/hepatoblastoma-the-liver-tumour-of-early-childhood/