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Published on: March 7, 2017
A comprehensive review of paediatric low-grade diffuse glioma: pathology, molecular genetics and treatment
Scott Ryall1,2, Uri Tabori1,3, Cynthia Hawkins4,5,6
1The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G1X8, Canada.
Insights
Paediatric grade II diffuse gliomas are distinct from adult forms, rarely progressing or harboring IDH mutations. This review covers their diagnosis, molecular features, and treatment options for pediatric brain tumors.
Area of Science:
- Pediatric neuro-oncology
- Central nervous system neoplasms
- Molecular pathology of pediatric brain tumors
Background:
- Gliomas are the most common pediatric central nervous system neoplasms.
- Paediatric low-grade gliomas include World Health Organization grade I and II.
- Pediatric grade II diffuse astrocytomas are clinically and molecularly distinct from adult types.
Purpose of the Study:
- To review the clinical, histologic, and molecular features of pediatric grade II diffuse glioma.
- To highlight diagnostic criteria, prevalence, and molecular characteristics.
- To discuss current therapeutic options for pediatric diffuse gliomas.
Main Methods:
- Review of clinical data.
- Histologic examination.
- Molecular feature analysis.
- Diagnostic criteria evaluation.
- Therapeutic option assessment.
Main Results:
- Pediatric grade II diffuse gliomas exhibit distinct clinical and molecular profiles compared to adults.
- These tumors rarely progress to higher-grade lesions.
- IDH mutations are infrequently found in pediatric diffuse gliomas.
Conclusions:
- Understanding the unique characteristics of pediatric grade II diffuse glioma is crucial for accurate diagnosis and treatment.
- Further research into targeted therapies based on specific molecular features is warranted.
- This review provides a comprehensive overview for clinicians managing these pediatric brain tumors.
Abstract:
Gliomas are the most common central nervous system neoplasms affecting children and can be both high- and low-grade. Paediatric low-grade glioma may be either World Health Organization grade I or grade II. Despite being classified as grade II diffuse astrocytoma, these neoplasms arising in children are distinct clinically and molecularly from their adult counterparts. They do not tend to progress to higher grade lesions and only rarely harbour an IDH mutation. Here, we review the clinical, histologic and molecular features of paediatric grade II diffuse glioma, highlighting their diagnostic criteria, prevalence across brain locations, their most common molecular features and how to test for them, and lastly the current status of therapeutic options available for their treatment.
