Open AccessMolecular Mechanisms of Hepatoblastoma
Author(s) -
Yi Zhang,
A Solinas,
Stefano Cairo,
Matthias Evert,
Xin Chen,
Diego F. Calvisi
Publication year - 2021
Publication title -
seminars in liver disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.874
H-Index - 126
eISSN - 1098-8971
pISSN - 0272-8087
DOI - 10.1055/s-0040-1722645
Subject(s) - hepatoblastoma , pi3k/akt/mtor pathway , cancer research , wnt signaling pathway , medicine , pathogenesis , disease , bioinformatics , biology , signal transduction , genetics
Hepatoblastoma (HB) is the predominant primary liver tumor in children. While the prognosis is favorable when the tumor can be resected, the outcome is dismal for patients with progressed HB. Therefore, a better understanding of the molecular mechanisms responsible for HB is imperative for early detection and effective treatment. Sequencing analysis of human HB specimens unraveled the pivotal role of Wnt/β-catenin pathway activation in this disease. Nonetheless, β-catenin activation alone does not suffice to induce HB, implying the need for additional alterations. Perturbations of several pathways, including Hippo, Hedgehog, NRF2/KEAP1, HGF/c-Met, NK-1R/SP, and PI3K/AKT/mTOR cascades and aberrant activation of c-MYC, n-MYC, and EZH2 proto-oncogenes, have been identified in HB, although their role requires additional investigation. Here, we summarize the current knowledge on HB molecular pathogenesis, the relevance of the preclinical findings for the human disease, and the innovative therapeutic strategies that could be beneficial for the treatment of HB patients.