Hepatoblastoma and Pediatric Liver Tumors (Volume II)

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Pediatric Oncology".

Deadline for manuscript submissions: 10 December 2024 | Viewed by 1266

Special Issue Editors


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Guest Editor
Department of Pathology and Cell Biology, Columbia University Irving ​Medical Center, New York Presbyterian Hospital, New York, NY, USA
Interests: hepatoblastoma; hepatocellular carcinoma; molecular tumor profiling; personalized genomics

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Guest Editor
Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York Presbyterian Hospital, New York, NY, USA
Interests: pediatric and adult liver tumors and medical conditions; gastrointestinal tract cancer

E-Mail Website
Guest Editor
Department of Pediatrics, Division of Pediatric Hematology/Oncology/SCT, Columbia University Irving Medical Center, New York, NY, USA
Interests: immunooncology; immunotherapy; tumor microenvironment; pediatric solid tumors and brain tumors

Special Issue Information

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

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 submissions that pass pre-check are 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 semimonthly 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 2900 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 (1 paper)

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Research

12 pages, 2196 KiB  
Article
Hepatoblastoma Relapse—Findings from the German HB99 Trial and the German Liver Tumor Registry
by Rebecca Maxwell, Beate Häberle, Roland Kappler, Dietrich von Schweinitz, Mark Rassner, Julia von Frowein and Irene Schmid
Cancers 2024, 16(4), 696; https://doi.org/10.3390/cancers16040696 - 6 Feb 2024
Viewed by 892
Abstract
Survival rates for HB patients have improved; however, outcomes for patients who relapse remain poor. A retrospective review of information gathered for the HB99 study and the German Liver Tumor Registry identified 25 relapse patients (6.9%, 25/362). The median time from initial diagnosis [...] Read more.
Survival rates for HB patients have improved; however, outcomes for patients who relapse remain poor. A retrospective review of information gathered for the HB99 study and the German Liver Tumor Registry identified 25 relapse patients (6.9%, 25/362). The median time from initial diagnosis to first relapse was 13 months (range: 5–66 months). Two patients relapsed >36 months after initial diagnosis. A total of 68% (17/25) of relapses were metastatic, 24% local, and 8% combined. 67% of local relapses were alive at the last follow-up, in contrast to 53% of metastatic and 0% of combined relapses. At the last follow-up, 73% (8/11) of patients with lung relapses were still alive (0/4 with peritoneal, 1/2 with CNS involvement). A total of 20% of the patients had AFP-negative relapses, 64% of the relapse patients achieved a second complete remission, 69% were still in complete second remission at the last follow-up (median FU of 66 months), and 83% (5/6) of irinotecan-naïve patients who received relapse treatment including irinotecan were in second complete remission at the last follow-up. The 3-year overall survival/event-free survival from relapse was 63%/48% respectively. There is a good chance that HB patients will achieve a second remission despite a first relapse. However, patients who suffer further relapses tend to have a poorer prognosis. Full article
(This article belongs to the Special Issue Hepatoblastoma and Pediatric Liver Tumors (Volume II))
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