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Cancers
Special Issues
Brain Tumour in Infancy: Pathology, Genetics and Therapeutic Approaches
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Brain Tumour in Infancy: Pathology, Genetics and Therapeutic Approaches

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

Deadline for manuscript submissions: closed (28 February 2022) | Viewed by 11267

Special Issue Editor

Dr. Maria Luisa Garre

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Guest Editor
IRCCS Istituto Giannina Gaslini-Ospedale Pediatrico, Genoa, Italy
Interests: brain tumors; late effects; epidemiology of brain tumours; targeted therapies in brain tumors

Special Issue Information

Dear colleagues,

The treatment of brain tumors (BTs) in young children (YC) (less than 3–5 years of age) in the last decade has been a special field of interest for clinical investigation and research, since infants affected by BT are an ideal experimental group of patients. New treatment strategies (intensive induction, myeloablative and intraventricular chemotherapy) have been tested on these patients in an effort to avoid or delay conventional irradiation. New irradiation techniques such as proton therapy have been more extensively adopted and accepted by cooperative trials to decrease the risk of severe late effects (SMNs) in treated patients. The vast amount of knowledge acquired from pathological and biological studies is increasingly identifying special biological features of brain tumors in YC in almost all tumor entities, either malignant  (embryonal tumors, ependymomas, high-grade gliomas) or benign (low-grade gliomas); new generation treatment “baby protocols” could now incorporate phenotypic and genotypic parameters in patient stratification. This Special Issue on this topic aims to focus on advances and controversies of treatment of YC with BT with particular regard to translation of biological and genetic information on treatment strategies and on the impact of late effects, as late effects of cancer treatment potentially correlate to a different genetic susceptibility.

Dr. Maria Luisa Garre
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 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 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

  • conventional and myeloablative chemotherapy
  • infants
  • late effects
  • embryonal tumor
  • ependymoma
  • high and low grade gliomas
  • familial cancer syndromes
  • proton therapy
  • methylation profile
  • tailored therapies

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Published Papers (2 papers)

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Research

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15 pages, 2325 KiB  
Article
Identification and Functional Analysis of a Novel CTNNB1 Mutation in Pediatric Medulloblastoma
by Lide Alaña, Caroline E. Nunes-Xavier, Laura Zaldumbide, Idoia Martin-Guerrero, Lorena Mosteiro, Piedad Alba-Pavón, Olatz Villate, Susana García-Obregón, Hermenegildo González-García, Raquel Herraiz, Itziar Astigarraga, Rafael Pulido and Miguel García-Ariza
Cancers 2022, 14(2), 421; https://doi.org/10.3390/cancers14020421 - 14 Jan 2022
Cited by 1 | Viewed by 3701
Abstract
Medulloblastoma is the primary malignant tumor of the Central Nervous System (CNS) most common in pediatrics. We present here, the histological, molecular, and functional analysis of a cohort of 88 pediatric medulloblastoma tumor samples. The WNT-activated subgroup comprised 10% of our cohort, and [...] Read more.
Medulloblastoma is the primary malignant tumor of the Central Nervous System (CNS) most common in pediatrics. We present here, the histological, molecular, and functional analysis of a cohort of 88 pediatric medulloblastoma tumor samples. The WNT-activated subgroup comprised 10% of our cohort, and all WNT-activated patients had exon 3 CTNNB1 mutations and were immunostained for nuclear β-catenin. One novel heterozygous CTNNB1 mutation was found, which resulted in the deletion of β-catenin Ser37 residue (ΔS37). The ΔS37 β-catenin variant ectopically expressed in U2OS human osteosarcoma cells displayed higher protein expression levels than wild-type β-catenin, and functional analysis disclosed gain-of-function properties in terms of elevated TCF/LEF transcriptional activity in cells. Our results suggest that the stabilization and nuclear accumulation of ΔS37 β-catenin contributed to early medulloblastoma tumorigenesis. Full article
(This article belongs to the Special Issue Brain Tumour in Infancy: Pathology, Genetics and Therapeutic Approaches)
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Review

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18 pages, 1223 KiB  
Review
Glioma Stem Cells in Pediatric High-Grade Gliomas: From Current Knowledge to Future Perspectives
by Marc-Antoine Da-Veiga, Bernard Rogister, Arnaud Lombard, Virginie Neirinckx and Caroline Piette
Cancers 2022, 14(9), 2296; https://doi.org/10.3390/cancers14092296 - 4 May 2022
Cited by 16 | Viewed by 6675
Abstract
In children, high-grade gliomas (HGG) and diffuse midline gliomas (DMG) account for a high proportion of death due to cancer. Glioma stem cells (GSCs) are tumor cells in a specific state defined by a tumor-initiating capacity following serial transplantation, self-renewal, and an ability [...] Read more.
In children, high-grade gliomas (HGG) and diffuse midline gliomas (DMG) account for a high proportion of death due to cancer. Glioma stem cells (GSCs) are tumor cells in a specific state defined by a tumor-initiating capacity following serial transplantation, self-renewal, and an ability to recapitulate tumor heterogeneity. Their presence was demonstrated several decades ago in adult glioblastoma (GBM), and more recently in pediatric HGG and DMG. In adults, we and others have previously suggested that GSCs nest into the subventricular zone (SVZ), a neurogenic niche, where, among others, they find shelter from therapy. Both bench and bedside evidence strongly indicate a role for the GSCs and the SVZ in GBM progression, fostering the development of innovative targeting treatments. Such new therapeutic approaches are of particular interest in infants, in whom standard therapies are often limited due to the risk of late effects. The aim of this review is to describe current knowledge about GSCs in pediatric HGG and DMG, i.e., their characterization, the models that apply to their development and maintenance, the specific signaling pathways that may underlie their activity, and their specific interactions with neurogenic niches. Finally, we will discuss the clinical relevance of these observations and the therapeutic advantages of targeting the SVZ and/or the GSCs in infants. Full article
(This article belongs to the Special Issue Brain Tumour in Infancy: Pathology, Genetics and Therapeutic Approaches)
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