Indian Journal of Pathology and Microbiology
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Year : 2022  |  Volume : 65  |  Issue : 5  |  Page : 50-58

Paediatric type diffuse high grade gliomas in the WHO CNS5 classification: What the pathologist needs to know?

Department of Pathology, Tata Memorial Hospital and ACTREC, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India

Correspondence Address:
Sridhar Epari
8th Floor, Annexe Building, Tata Memorial Hospital, Dr E. Borges Road, Parel, Mumbai – 400 012, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_1202_21

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Pediatric-type of diffuse high-grade gliomas (HGG) are classified as a distinct group in the current fifth edition of WHO classification. This group of high-grade tumors is no more called as glioblastoma (GBM), which has been reserved for adult isocitrate dehydrogenase (IDH)-wild type HGG. These tumors are uncommon as compared to embryonal tumors and low-grade gliomas (LGG). Pediatric-type of diffuse HGG biologically differs from their adult counterparts in that they are therapeutically less sensitive to alkylating chemotherapies. They comprise a heterogeneous group of molecularly defined tumors – predominantly histone gene altered, less common receptor tyrosine kinase (RTK)-mediated, and syndrome-associated. This review provides an overview of these uncommon tumors and discusses the diagnostic approach of this heterogeneous group of tumors.

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