Molecular Mechanisms of Hepatoblastoma

Zhang, Yi and Solinas, Antonio and Cairo, Stefano and Evert, Matthias and Chen, Xin and Calvisi, Diego F. (2021) Molecular Mechanisms of Hepatoblastoma. SEMINARS IN LIVER DISEASE, 41 (01). pp. 28-41. ISSN 0272-8087, 1098-8971

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Abstract

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/beta-catenin pathway activation in this disease. Nonetheless, beta-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.

Item Type: Article
Uncontrolled Keywords: PEDIATRIC LIVER-TUMOR; PRIMARY HEPATIC MALIGNANCIES; BECKWITH-WIEDEMANN SYNDROME; PURE FETAL HISTOLOGY; BETA-CATENIN; WNT/BETA-CATENIN; CELL-PROLIFERATION; SIGNALING PATHWAY; CANONICAL WNT; IN-VITRO; hepatoblastoma; Wnt; β -catenin; Hippo; NRF2
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Pathologie
Depositing User: Petra Gürster
Date Deposited: 17 Jan 2023 06:17
Last Modified: 17 Jan 2023 06:17
URI: https://pred.uni-regensburg.de/id/eprint/47052

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