Overview

NCI Definition: A malignant liver neoplasm that occurs almost exclusively in infants, although isolated cases in older children and adults have been reported. Grossly, hepatoblastoma is solid, well circumscribed, and more often solitary than multiple. Microscopically, most of the tumors are composed exclusively of immature hepatocytic elements. About a fourth of hepatoblastomas contain a stromal component that may be undifferentiated or develop into bone or cartilage. The treatment of choice for hepatoblastoma is surgical excision with adjuvant therapy. Liver transplantation is being increasingly used as well. [1]

Hepatoblastomas most frequently harbor alterations in CTNNB1 [2].

Most Commonly Altered Genes in Hepatoblastoma

CTNNB1 Mutation are the most common alterations in hepatoblastoma [2].

Top Alterations in Hepatoblastoma

Significant Genes in Hepatoblastoma

AXL +

MDM2 +

MDM4 +

MET +

PPM1D +

RET +

SMARCA4 +

SMARCB1 +

TET2 +

Disease Details

Synonyms
HEPATOBLASTOMA, MALIGNANT, PEDIATRIC Liver hepatoblastoma, Pediatric Embryonal Hepatoma, HBL, Pediatric Hepatoblastoma
Parent(s)
Primary Malignant Liver Neoplasm
Children
Small Cell Undifferentiated Hepatoblastoma, Macrotrabecular Hepatoblastoma, and Mixed Epithelial and Mesenchymal Hepatoblastoma
OncoTree Name
Hepatoblastoma
OncoTree Code
LIHB

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.