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Paediatric-type diffuse low-grade gliomas: A pictorial review
Krishanthi Nilushi Hewawasam Wadige1, Robert Goetti2
1Provisional fellow in Paediatric Radiology, Department of Medical Imaging, Children's Hospital at Westmead, NSW, Australia.
Insights
The WHO CNS5 classification distinguishes paediatric low-grade gliomas (pDLGGs) from high-grade types. This review highlights MRI findings for pDLGGs, aiding radiologists in diagnosing these distinct neoplasms.
Area of Science:
- Neuro-oncology
- Molecular diagnostics
- Paediatric neurosurgery
Background:
- The WHO CNS5 classification refines brain and spinal cord tumour diagnosis, emphasizing molecular markers.
- Paediatric diffuse gliomas are distinct from adult types in genetics, prognosis, and treatment.
- Paediatric low-grade gliomas (pDLGGs) are now recognized as separate entities from high-grade gliomas (pDHGGs).
Purpose of the Study:
- To provide an image-rich overview of pDLGGs based on the WHO CNS5 classification.
- To familiarize radiologists with the imaging hallmarks of pDLGGs.
- To aid in the differential diagnosis of paediatric brain tumours.
Main Methods:
- Pictorial review of MRI findings in pDLGGs.
- Emphasis on typical and atypical imaging features.
- Correlation with WHO CNS5 diagnostic criteria.
Main Results:
- pDLGGs encompass four distinct tumour types, three newly recognized.
- Molecular diagnostics are crucial for differentiating these entities.
- Familiarity with imaging findings is essential for accurate diagnosis and management.
Conclusions:
- The WHO CNS5 classification introduces significant changes in paediatric glioma diagnosis.
- Radiologists must understand the imaging characteristics of pDLGGs for effective clinical practice.
- Accurate diagnosis of pDLGGs impacts treatment strategies and patient outcomes.
Abstract:
The fifth edition of the World Health Organization (WHO) Classification of Tumours of the Central Nervous System (WHO CNS5) introduces a revised framework for classifying brain and spinal cord tumours, emphasizing the crucial role of molecular diagnostics in accurately identifying neoplasms. A key advancement in this edition is the clear distinction between paediatric-type diffuse low-grade gliomas (pDLGGs) and paediatric-type diffuse high-grade gliomas (pDHGGs) as separate tumour entities. Although histologically and morphologically similar, paediatric diffuse gliomas differ significantly from their adult counterparts in terms of molecular genetics, prognosis, and treatment approaches. The pDLGG category comprises four tumour types: diffuse astrocytoma with MYB- or MYBL1-alterations, angiocentric glioma, polymorphous low-grade neuroepithelial tumour of the young (PLNTY), and diffuse low-grade glioma with MAPK pathway alterations-three of which have been newly recognized. As imaging plays a critical role in the initial diagnosis, surgical planning, and post-treatment monitoring, it is essential for radiologists to be familiar with the imaging hallmarks that distinguish pDLGGs from other paediatric brain tumours, including high-grade gliomas and non-glial neoplasms. This pictorial review aims to provide an updated, image-rich overview of paediatric diffuse low-grade gliomas, emphasizing their typical and atypical imaging findings in MRI, offering valuable diagnostic insights for clinical practitioners and Radiologist.
