Special Issue "Hepatoblastoma and Pediatric Liver Tumors"

A special issue of Cancers (ISSN 2072-6694).

Deadline for manuscript submissions: 30 June 2019

Special Issue Editor

Guest Editor
Dr. Helen Remotti

Associate Professor of Pathology and Cell Biology Columbia University Medical Center, New York Presbyterian Hospital 630 West 168th St., VC14-215, New York, NY 10032
Website | E-Mail
Interests: hepatoblastoma; hepatocellular carcinoma; molecular tumor profiling; personalized genomics

Special Issue Information

Dear Colleagues,

Primary liver cancers are rare in children, with hepatoblastomas representing the most common liver tumor in children, 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 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 be comprised of 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 represents an aggressive unrelated tumor—the malignant rhabdoid tumor, that can be diagnosed by molecular confirmation of SMARCB1 deletions and lack of protein expression of INI-1, a member of the chromatin remodeling SWI/SNF complex. With increased awareness of the molecular classification of liver tumors, there is a need for standard classification of liver tumors, incorporating routine use of immunostaining and molecular assays to correctly classify rare malignant liver tumors, that may benefit by 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, that currently are not therapeutically targetable. Transcriptome and methylation studies may be useful in classifying tumors, but limited studies have been performed and they are not utilized in clinical practice to date.

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

Dr. Helen Remotti
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All papers will be peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cancers is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 1800 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • Hepatoblastoma
  • Personalized Genomics
  • Pediatric liver tumor
  • Hepatocellular carcinoma
  • Precision Medicine

Published Papers (3 papers)

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Research

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Open AccessArticle Longitudinal Monitoring of Alpha-Fetoprotein by Dried Blood Spot for Hepatoblastoma Screening in Beckwith–Wiedemann Syndrome
Received: 11 November 2018 / Revised: 21 December 2018 / Accepted: 11 January 2019 / Published: 14 January 2019
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Abstract
Background: Hepatoblastoma screening in the Beckwith–Wiedemann spectrum (BWSp) is currently based on measuring a specific serum marker alpha-fetoprotein (αFP) every three months until the fourth birthday. Frequent blood draws can be a burden for patients and their families. Methods: We have developed a [...] Read more.
Background: Hepatoblastoma screening in the Beckwith–Wiedemann spectrum (BWSp) is currently based on measuring a specific serum marker alpha-fetoprotein (αFP) every three months until the fourth birthday. Frequent blood draws can be a burden for patients and their families. Methods: We have developed a less invasive alternative testing method based on measuring αFPs from dried blood spots (DBS). The method was validated with 259 simultaneous plasma and DBS αFP measurements in 171 children (132 controls and 39 patients with BWSp). Results: The DBS and plasma measurements overlapped across the wide range of αFP concentrations independent of patient age (p < 0.0001), demonstrating the utility of this method for longitudinal monitoring. Occasional differences between measurements by the two techniques fell within standard laboratory error and would not alter clinical management. Conclusions: This novel method shows consistent overlap with the traditional blood draws, thereby demonstrating its utility for hepatoblastoma screening in this setting and alleviating the burden of frequent blood draws. This also may help increase patient compliance and reduce costs of health care screening. The DBS-based method for the measurement of cancer biomarkers may also be applied to several other chronic diseases with increased risks of αFP-producing liver tumors. Full article
(This article belongs to the Special Issue Hepatoblastoma and Pediatric Liver Tumors)
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Review

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Open AccessReview The Role of MicroRNAs in Hepatoblastoma Tumors
Cancers 2019, 11(3), 409; https://doi.org/10.3390/cancers11030409
Received: 30 January 2019 / Revised: 15 March 2019 / Accepted: 19 March 2019 / Published: 22 March 2019
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Abstract
Hepatoblastoma is the most common hepatic malignancy during childhood. However, little is still known about the molecular mechanisms that govern the development of this disease. This review is focused on the recent advances regarding the study of microRNAs in hepatoblastoma and their substantial [...] Read more.
Hepatoblastoma is the most common hepatic malignancy during childhood. However, little is still known about the molecular mechanisms that govern the development of this disease. This review is focused on the recent advances regarding the study of microRNAs in hepatoblastoma and their substantial contribution to improv our knowledge of the pathogenesis of this disease. We show here that miRNAs represent valuable tools to identify signaling pathways involved in hepatoblastoma progression as well as useful biomarkers and novel molecular targets to develop alternative therapeutic strategies in this disease. Full article
(This article belongs to the Special Issue Hepatoblastoma and Pediatric Liver Tumors)
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Open AccessReview Mechanisms of Anticancer Drug Resistance in Hepatoblastoma
Cancers 2019, 11(3), 407; https://doi.org/10.3390/cancers11030407
Received: 7 February 2019 / Revised: 12 March 2019 / Accepted: 18 March 2019 / Published: 22 March 2019
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Abstract
The most frequent liver tumor in children is hepatoblastoma (HB), which derives from embryonic parenchymal liver cells or hepatoblasts. Hepatocellular carcinoma (HCC), which rarely affects young people, causes one fourth of deaths due to cancer in adults. In contrast, HB usually has better [...] Read more.
The most frequent liver tumor in children is hepatoblastoma (HB), which derives from embryonic parenchymal liver cells or hepatoblasts. Hepatocellular carcinoma (HCC), which rarely affects young people, causes one fourth of deaths due to cancer in adults. In contrast, HB usually has better prognosis, but this is still poor in 20% of cases. Although more responsive to chemotherapy than HCC, the failure of pharmacological treatment used before and/or after surgical resection is an important limitation in the management of patients with HB. To advance in the implementation of personalized medicine it is important to select the best combination among available anti-HB drugs, such as platinum derivatives, anthracyclines, etoposide, tyrosine-kinase inhibitors, Vinca alkaloids, 5-fluorouracil, monoclonal antibodies, irinotecan and nitrogen mustards. This requires predicting the sensitivity to these drugs of each tumor at each time because, it should be kept in mind, that cancer chemoresistance is a dynamic process of Darwinian nature. For this goal it is necessary to improve our understanding of the mechanisms of chemoresistance involved in the refractoriness of HB against the pharmacological challenge and how they evolve during treatment. In this review we have summarized the current knowledge on the multifactorial and complex factors responsible for the lack of response of HB to chemotherapy. Full article
(This article belongs to the Special Issue Hepatoblastoma and Pediatric Liver Tumors)
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