hepatoblastoma

Introduction

malignant embryonal neoplasm

Epidemiology

• most frequent liver tumor in children
• 4% present at birth
• 68% first two years
• 90% by age 5 years

Pathology

• variable histological patterns
• pure fetal epithelial differentiation: ~ 1/3 of cases resemble hepatocytes in developing fetal liver
• combined fetal and embryonal epithelial
• macrotrabecular
• small cell undifferentiated
• mixed epithelial & mesenchymal
  • mesenchymal components include fibrous tissue, osteoid- like tissue & cartilagenous tissue
• mixed with teratoid features
  • additional possibile features include striated muscle, bone, mucinous epithelium, squamous epithelium, melanin pigment

Genetics

• associated with mutations in CTNNB1

Laboratory

• alpha fetoprotein elevated in 90%
• anemia 70%

thrombocytopenia 50%

More general terms

• liver cancer; hepatobiliary carcinoma
• embryonal neoplasm

More specific terms

• hepatoblastoma [TP53/SER241PHE]