As a relevant element of novelty, the fifth CNS WHO Classification highlights the distinctive pathobiology underlying gliomas arising primarily in children by recognizing for the first time the families of paediatric-type diffuse gliomas, both high-grade and low-grade. This review will focus on the family of paediatric-type diffuse high-grade gliomas, which includes four tumour types: 1) Diffuse midline glioma H3 K27-altered; 2) Diffuse hemispheric glioma H3 G34-mutant; 3) Diffuse paediatric-type high-grade glioma H3-wildtype and IDH-wildtype; and 4) Infant-type hemispheric glioma. The essential and desirable diagnostic criteria as well as the entities entering in the differential will be discussed for each tumour type. A special focus will be given on the issues encountered in the daily practice, especially regarding the diagnosis of the diffuse paediatric-type high-grade glioma H3-wildtype and IDH-wildtype. The advantages and the limits of the multiple molecular tests which may be utilised to define the entities of this tumour family will be evaluated in each diagnostic context.
Paediatric-type diffuse high-grade gliomas in the 5th CNS WHO Classification / Gianno, Francesca; Giovannoni, Isabella; Cafferata, Barbara; Diomedi-Camassei, Francesca; Minasi, Simone; Barresi, Sabina; Buttarelli, Francesca Romana; Alesi, Viola; Cardoni, Antonello; Antonelli, Manila; Puggioni, Chiara; Stefania Colafati, Giovanna; Carai, Andrea; Vinci, Maria; Mastronuzzi, Angela; Miele, Evelina; Alaggio, Rita; Giangaspero, Felice; Rossi, Sabrina. - In: PATHOLOGICA. - ISSN 1591-951X. - 114:6(2022), pp. 422-435. [10.32074/1591-951X-830]
Paediatric-type diffuse high-grade gliomas in the 5th CNS WHO Classification
Francesca GiannoPrimo
;Simone Minasi;Francesca Romana Buttarelli;Manila Antonelli;Rita Alaggio;Felice GiangasperoPenultimo
;
2022
Abstract
As a relevant element of novelty, the fifth CNS WHO Classification highlights the distinctive pathobiology underlying gliomas arising primarily in children by recognizing for the first time the families of paediatric-type diffuse gliomas, both high-grade and low-grade. This review will focus on the family of paediatric-type diffuse high-grade gliomas, which includes four tumour types: 1) Diffuse midline glioma H3 K27-altered; 2) Diffuse hemispheric glioma H3 G34-mutant; 3) Diffuse paediatric-type high-grade glioma H3-wildtype and IDH-wildtype; and 4) Infant-type hemispheric glioma. The essential and desirable diagnostic criteria as well as the entities entering in the differential will be discussed for each tumour type. A special focus will be given on the issues encountered in the daily practice, especially regarding the diagnosis of the diffuse paediatric-type high-grade glioma H3-wildtype and IDH-wildtype. The advantages and the limits of the multiple molecular tests which may be utilised to define the entities of this tumour family will be evaluated in each diagnostic context.| File | Dimensione | Formato | |
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