Hepatoblastoma

Although malignant tumors of the liver are rare during childhood, hepatoblastoma is the most common liver cancer in children, usually diagnosed during the first 3 years of life. These tumors are thought to arise from hepatic progenitors or hepatoblasts. The most frequent genetic aberrations (70-90%) in hepatoblastoma occur in genes involved in the Wnt signaling pathway. The Wnt signaling pathway is activated often by acquired activating mutations of the beta-catenin (CTNNB1) gene, and less commonly through constitutional mutations of the APC gene or somatic mutations of other genes in the pathway.

Cancer

CTNNB1 (mutation) [HSA:1499] [KO:K02105]
APC (mutation) [HSA:324] [KO:K02085]
AXIN2 (mutation) [HAS:8313] [KO:K04385]

ICD-O-3.1:8970/3 Hepatoblastoma
ICD-O-3.1:8970/3 Hepatoblastoma, epithelioid
ICD-O-3.1:8970/3 Hepatoblastoma, mixed epithelial-mesenchymal

PMID:22807084

Tomlinson GE, Kappler R

Genetics and epigenetics of hepatoblastoma.

Pediatr Blood Cancer 59:785-92 (2012)
DOI:10.1002/pbc.24213

PMID:29251405

Yang A, Sisson R, Gupta A, Tiao G, Geller JI

Germline APC mutations in hepatoblastoma.

Pediatr Blood Cancer 65:e26892 (2018)
DOI:10.1002/pbc.26892

PMID:27938502

Bell D, Ranganathan S, Tao J, Monga SP

Novel Advances in Understanding of Molecular Pathogenesis of Hepatoblastoma: A Wnt/beta-Catenin Perspective.

Gene Expr 17:141-154 (2017)
DOI:10.3727/105221616X693639

PMID:24322718

Czauderna P, Lopez-Terrada D, Hiyama E, Haberle B, Malogolowkin MH, Meyers RL

Hepatoblastoma state of the art: pathology, genetics, risk stratification, and chemotherapy.

Curr Opin Pediatr 26:19-28 (2014)
DOI:10.1097/MOP.0000000000000046

PMID:22648938

Lopez-Terrada D, Zimmermann A

Current issues and controversies in the classification of pediatric hepatocellular tumors.

Pediatr Blood Cancer 59:780-4 (2012)
DOI:10.1002/pbc.24214