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  • Updated 08.23.2023
  • Released 04.29.2003
  • Expires For CME 08.23.2026

Pediatric-type diffuse high-grade gliomas (excluding diffuse midline gliomas)



Pediatric-type diffuse high-grade gliomas continue to be one of the most difficult types of brain tumors to treat in children, given their infiltrative nature and low probability of successful gross total resection. In this article, the authors present a comprehensive review of the basic principles and practices underlying the causes, diagnosis, treatment, and outcomes of pediatric-type diffuse high-grade glioma. The authors also present updated therapeutic and molecular research advancements in these childhood tumors.

Key points

• Molecular characterization of pediatric-type diffuse high-grade glioma is explored.

• There are four distinct subtypes of pediatric-type diffuse high-grade glioma with different, distinct characteristics.

• The diffuse nature of these tumors makes gross total resection impossible, which contributes greatly to the poor prognosis.

• Survival remains poor for most of these tumors; however, new clinical trials are being investigated.

Historical note and terminology

Brain tumor classification began in the 1920s. The proposed classification system for gliomas was based on the idea that gliomas originated from central nervous system cells arrested at various stages of development (02). Recognizing that brain tumors contained heterogeneous cell populations, gliomas were classified based on the morphological appearance and presumed histogenesis of the predominant cell type. Most classification schemes have been constructed to various degrees around the conceptual framework introduced by Bailey and Cushing (43; 28). Other classifications have proposed that glial cells become progressively more anaplastic rather than undergo a single neoplastic transformation to malignant glioma (45). This concept has led to the proposal to simplify brain tumor classification to reflect the degree of anaplasia present by grading the tumors from grade I (benign) to grade IV (malignant). However, with advancements in tumor biology genetics, it is now known that histopathology alone cannot provide prognostic value to the diagnosis. As a result, in 2016, an international collaboration of renowned scientists and clinicians revised the current World Health Organization classifications by including molecular parameters (32).

This was further emphasized in the most recent version of the WHO Classification of Tumors of the Central Nervous System, version 5, which was released in 2021 and subdivided pediatric-type diffuse high-grade glioma into four distinct subtypes: diffuse midline glioma; H3 K27-altered (which will be discussed separately), diffuse hemispheric glioma; H3 G34-mutant, diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype; and infant-type hemispheric glioma (33). An account of the historical evolution of glioma terminology has been previously provided in much greater detail (54).

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ISSN: 2831-9125