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Published on: March 7, 2017
Paediatric-type diffuse low-grade gliomas: a clinically and biologically distinct group of tumours with a favourable
Viscardo Paolo Fabbri1,2, Chiara Caporalini3, Sofia Asioli1,4
1Department of Biomedical and Neuromotor Sciences (DIBINEM), Alma Mater Studiorum University of Bologna, Bologna, Italy.
Insights
The World Health Organization 2021 classification separates adult and pediatric gliomas. This study examines newly recognized pediatric-type gliomas in younger adults and proposes an integrated diagnostic approach for these distinct tumor groups.
Area of Science:
- Neuro-oncology
- Molecular Pathology
- Pediatric Oncology
Background:
- The 2021 WHO classification distinguishes adult-type and pediatric-type diffuse gliomas based on clinical and molecular features.
- Paediatric-type gliomas can occur in younger adults, and adult-type gliomas can occur in children, necessitating careful classification.
- Recognized entities of diffuse low-grade pediatric glioma include MYB/MYBL1-altered diffuse astrocytoma, polymorphous glioma, angiocentric glioma, and MAPK pathway-altered glioma.
Purpose of the Study:
- To examine newly recognized entities of diffuse pediatric-type gliomas according to WHO diagnostic criteria.
- To propose an integrated diagnostic approach for differentiating these distinct tumor groups.
- To clarify the classification of gliomas that may present outside typical age demographics.
Main Methods:
- Review of WHO 2021 diagnostic criteria for central nervous system cancers.
- Analysis of clinical and molecular characteristics of pediatric-type gliomas.
- Development of an integrated diagnostic strategy for glioma classification.
Main Results:
- Identification and characterization of specific pediatric-type glioma entities.
- Understanding of overlapping age distributions for adult-type and pediatric-type gliomas.
- Establishment of criteria for distinguishing these clinically and biologically distinct tumor groups.
Conclusions:
- The integrated diagnostic approach aids in separating pediatric-type gliomas from adult-type gliomas.
- Accurate classification is crucial for understanding the distinct clinical and biological behavior of these gliomas.
- This work supports precise diagnosis and management of pediatric and young adult gliomas.
Abstract:
The WHO 2021 classification of central nervous system cancers distinguishes diffuse gliomas that arise in adults (referred to as the "adult type") and those that arise in children (defined as "paediatric") based on clinical and molecular characteristics."). However, paediatric-type gliomas may occasionally be present in younger adults and occasionally adult-type gliomas may occur in children. Diffuse low-grade paediatric glioma includes diffuse astrocytoma altered by MYB or MYBL1, low-grade polymorphic juvenile neuroepithelial tumour, angiocentric glioma, and diffuse low-grade glioma with an altered MAPK pathway. Here, we examine these newly recognised entities according to WHO diagnostic criteria and propose an integrated diagnostic approach that can be used to separate these clinically and biologically distinct tumor groups.
