TY - JOUR
T1 - Paediatric-type diffuse high-grade gliomas in the 5th CNS WHO Classification
AU - Gianno, F.
AU - Giovannoni, I.
AU - Cafferata, B.
AU - Diomedi-Camassei, F.
AU - Minasi, S.
AU - Barresi, S.
AU - Buttarelli, F. R.
AU - Alesi, V.
AU - Cardoni, A.
AU - Antonelli, M.
AU - Puggioni, C.
AU - Colafati, G. S.
AU - Carai, A.
AU - Vinci, M.
AU - Mastronuzzi, Angela
AU - Miele, E.
AU - Alaggio, R.
AU - Giangaspero, F.
AU - Rossi, S.
PY - 2022
Y1 - 2022
N2 - 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.
AB - 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.
KW - DMG with EZHIP overexpression
KW - ETV6-NTRK3
KW - MEF2D-NTRK1
KW - MYC amplification
KW - MYCN amplification
KW - PDGFRA amplification
KW - RTK fusions
KW - ZCCHC8-ROS1
KW - diffuse hemispheric glioma H3 G34-mutant
KW - diffuse midline glioma K27M-altered
KW - diffuse paediatric-type high grade glioma H3-wildtype and IDH-wildtype
KW - infant-type hemispheric glioma
KW - infant-type hemispheric glioma with atypical location
KW - pHGG MYCN
KW - pHGG RTK1
KW - pHGG RTK2
KW - paediatric high-grade glioma
KW - radiation-induced gliomas
KW - DMG with EZHIP overexpression
KW - ETV6-NTRK3
KW - MEF2D-NTRK1
KW - MYC amplification
KW - MYCN amplification
KW - PDGFRA amplification
KW - RTK fusions
KW - ZCCHC8-ROS1
KW - diffuse hemispheric glioma H3 G34-mutant
KW - diffuse midline glioma K27M-altered
KW - diffuse paediatric-type high grade glioma H3-wildtype and IDH-wildtype
KW - infant-type hemispheric glioma
KW - infant-type hemispheric glioma with atypical location
KW - pHGG MYCN
KW - pHGG RTK1
KW - pHGG RTK2
KW - paediatric high-grade glioma
KW - radiation-induced gliomas
UR - https://publicatt.unicatt.it/handle/10807/329566
UR - https://www.scopus.com/inward/citedby.uri?partnerID=HzOxMe3b&scp=85144290593&origin=inward
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85144290593&origin=inward
U2 - 10.32074/1591-951X-830
DO - 10.32074/1591-951X-830
M3 - Article
SN - 1591-951X
VL - 114
SP - 422
EP - 435
JO - Pathologica
JF - Pathologica
IS - 6
ER -