The Advances in Molecular Biology of Hepatoblastoma: Implications for Diagnostic Pathology

Weiwei Chen, MD, PhD, Rafal Kozielski, MD, Charles LeVea, MD, PhD, Frank Chen, MD, PhD


As the most common pediatric liver malignancy, hepatoblastoma (HB) accounts for more than 90% of primary hepatic malignant tumors in children less than five years of age in the US, and its incidence has been increasing in the past decades. Despite extensive studies, the pathogenesis of HB remains to be elucidated. Multiple signaling pathways may be involved in the oncogenic process of HB. The best characterized pathways include the canonical Wnt/beta-catenin pathway, the hepatocyte growth factor (HGF)/c-Met signaling pathway, the Notch pathway and the Hedgehog pathway. In addition, signaling molecules associated with these signaling pathways have been shown to be potential novel tumor markers for HB. Preoperative chemotherapy is the current standard of care for HB. Highly sensitive and specific tumor markers are not only important for the accurate diagnosis of HB but are also essential for predicting its clinical behaviors and prognosis. This review summarizes the recent advances in the molecular aspects of HB with a focus on the pathogenic signaling pathways and tumor markers. Their implications for diagnostics and prognostics are also discussed from a pathologist’s point of view.


hepatoblastoma, pediatric liver malignancy, canonical Wnt/beta-catenin signaling pathway, hepatocyte growth factor (HGF)/c-Met signaling pathway, Notch signaling pathway, Hedgehog signaling pathway

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