The World Health Organization's 5th edition classification of Central Nervous System (CNS) tumors differentiates diffuse gliomas into adult and pediatric variants. Pediatric-type diffuse low-grade gliomas (pDLGGs) are distinct from adult gliomas in their molecular characteristics, biological behavior, clinical progression, and prognosis. Various molecular alterations identified in pDLGGs are crucial for treatment. There are four distinct entities of pDLGGs. All four of these tumor subtypes exhibit diffuse growth and share overlapping histopathological and imaging characteristics. Molecular analysis is essential for differentiating these lesions.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1007/s00381-024-06500-x | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Are Dinucleotide Alterations Definitional for Myxoid Glioneuronal Tumor? Report of p. K385L Mutation in a Neonatal High-Grade Glioma.
Pediatr Dev Pathol
December 2024
Department of Pathology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Tumors are increasingly defined by molecular alterations but approach to cases with discordant histologic and molecular features is unclear. Myxoid glioneuronal tumor (MGNT), histologically similar to dysembryoplastic neuroepithelial tumor (DNET), is characterized by dinucleotide mutations in gene (K385L or K385I). Here, we report K385L mutation in a neonatal high-grade glioma.
View Article and Find Full Text PDFDiffuse pediatric high-grade glioma of methylation-based RTK2A and RTK2B subclasses present distinct radiological and histomolecular features including Gliomatosis cerebri phenotype.
Acta Neuropathol Commun
November 2024
Department of Neuropathology, GHU Paris-Psychiatrie et Neurosciences, Sainte-Anne Hospital, 1, Rue Cabanis, 75014, Paris, France.
Diffuse pediatric-type high-grade gliomas (pedHGG), H3- and IDH-wildtype, encompass three main DNA-methylation-based subtypes: pedHGG-MYCN, pedHGG-RTK1A/B/C, and pedHGG-RTK2A/B. Since their first description in 2017 tumors of pedHGG-RTK2A/B have not been comprehensively characterized and clinical correlates remain elusive. In a recent series of pedHGG with a Gliomatosis cerebri (GC) growth pattern, an increased incidence of pedHGG-RTK2A/B (n = 18) was observed.
View Article and Find Full Text PDFRecent updates in pediatric diffuse glioma classification: insights and conclusions from the WHO 5 edition.
J Med Life
July 2024
Department of Pathology, All India Institute of Medical Sciences, Rajkot, Gujarat, India.
The World Health Organization (WHO) Central Nervous System (CNS) Tumors Classification 5 edition (2021) integrates both molecular and histopathological criteria for diagnosing glial tumors. This updated classification highlights significant differences between pediatric and adult gliomas in terms of molecular characteristics and prognostic implications. The 5 edition comprises a new category of pediatric-type diffuse low-grade glioma (PDLGG) and pediatric-type diffuse high-grade glioma (PDHGG), classified mainly based on genetic alterations and histopathological features.
View Article and Find Full Text PDFMYB/MYBL1-altered gliomas frequently harbor truncations and non-productive fusions in the MYB and MYBL1 genes.
Acta Neuropathol
October 2024
Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 10 Center Dr., Room 2S235, Bethesda, MD, 20892, USA.
Astrocytomas that harbor recurrent genomic alterations in MYB or MYBL1 are a group of Pediatric-type diffuse low-grade gliomas that were newly recognized in the 2021 WHO Classification of Tumors of the Central Nervous System. These tumors are described in the WHO classification as harboring fusions in MYB or MYBL1. In this report, we examine 14 consecutive cases in which a MYB or MYBL1 alteration was identified, each with diagnostic confirmation by genome-wide DNA methylation profiling (6 Angiocentric gliomas and 8 Diffuse astrocytomas, MYB- or MYBL1-altered), for their specific genomic alterations in these genes.
View Article and Find Full Text PDFEmerging and Biological Concepts in Pediatric High-Grade Gliomas.
Cells
September 2024
Centre for Cancer Research, Hudson Institute of Medical Research, Monash University, Clayton, VIC 3168, Australia.
Primary central nervous system tumors are the most frequent solid tumors in children, accounting for over 40% of all childhood brain tumor deaths, specifically high-grade gliomas. Compared with pediatric low-grade gliomas (pLGGs), pediatric high-grade gliomas (pHGGs) have an abysmal survival rate. The WHO CNS classification identifies four subtypes of pHGGs, including Grade 4 Diffuse midline glioma H3K27-altered, Grade 4 Diffuse hemispheric gliomas H3-G34-mutant, Grade 4 pediatric-type high-grade glioma H3-wildtype and IDH-wildtype, and infant-type hemispheric gliomas.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!