Hepatoblastoma and Pediatric Liver Tumors (Volume II)

Dear Colleagues,

This collection is the second edition of the previous Special Issue, "Hepatoblastoma and Pediatric Liver Tumors" (https://www.mdpi.com/journal/cancers/special_issues/Hepatoblastoma).

Primary liver cancers are rare in children, with hepatoblastomas representing the most common liver tumor in this group, comprising 1% of all pediatric cancers. Hepatoblastomas have an overall survival rate greater than 75%, which is largely related to the progress made in diagnosis and radiologic assessment using a standardized presurgical extent of disease (PRETEXT) staging system with the optimization of chemotherapeutic and surgical regimens. Fortunately, the majority of hepatoblastomas respond to treatment with cisplatin and the majority of tumors are resectable following chemotherapy. Liver transplantation is an alternative for PRETEXT stage I–III tumors involving essential vascular structures or for multifocal PRETEXT stage IV tumors. While outcomes have been good for standard-risk patients, the prognosis is poor for high-risk patients with advanced, metastatic or recurrent disease. It is still a challenge to optimize therapies for these children at highest risk.

Hepatoblastoma is an embryonal tumor that may show histologic heterogeneity with mixed epithelial and stromal components. The epithelial component may comprise fetal hepatocytes or more primitive embryonal hepatocytes. In light of molecular studies, it is now recognized that a large portion of small cell undifferentiated liver tumors (SCUDs) actually represent an aggressive unrelated tumor—the malignant rhabdoid tumor, which can be diagnosed by the molecular confirmation of SMARCB1 deletions and the lack of expression of the INI-1 protein, a member of the chromatin remodeling SWI/SNF complex. With increased awareness of the molecular classification of liver tumors, there is a need for the standard classification of liver tumors, incorporating the routine use of immunostaining and molecular assays to correctly classify rare malignant liver tumors, which  may benefit from individualized targeted therapy. To date, molecular studies performed on hepatoblastomas show recurrent mutations or deletions in CTNNB1, encoding beta catenin involved in the Wnt signaling pathway. Other mutations in genes that are involved in the Wnt signaling pathway such as APC, AXIN1 and AXIN2 have also been reported. Whole-exome studies have revealed a low frequency of somatic mutations in hepatoblastomas, which are not currently therapeutically targetable. Transcriptome and methylation studies may be useful in classifying tumors, but limited studies have been performed, and they have not yet been utilized in clinical practice.

This Special Issue will highlight the diverse challenges encountered in understanding the pathogenesis and the clinical management of pediatric liver tumors, with a primary focus on hepatoblastomas. Invited articles, both original research articles and reviews, will cover a wide range of topics, including, but not restricted to, the following: (1) Updates on the clinical management of pediatric liver tumors (hepatoblastomas), focusing on therapies for high-risk patients. (2) The utility of biomarkers for the stratification of patients into low- and high-risk groups. (3) Updates on the pathologic and molecular classification of hepatoblastomas. (4) Molecular studies distinguishing pediatric hepatocellular carcinoma versus hepatoblastoma and other unusual pediatric liver tumors. (5) The role of personalized genomics in discovering therapeutic targets and distinguishing between somatic and germline mutations.

We look forward to receiving your contributions.

Dr. Helen E. Remotti
Dr. Ladan Fazlollahi
Dr. Robyn Gartrell
Guest Editors

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• hepatoblastoma
• personalized genomics
• pediatric liver tumor
• hepatocellular carcinoma
• precision medicine