07.11.2017
6
Glioblastoma
31
• New pattern: Glioblastoma with primitive neuronal component
Pattern =Histological features without clear clinical significance
MYCN amp
Louiset al,
Acta Neuropath 2016
Diffuse midline glioma
32
• K27M mutations in histone H3 gene
H3F3A
defines a group
New diagnostic entity
Mutation-specific antibody
Louiset al,
Acta Neuropath 2016
Embryonal tumors
33
Greatest conceptual challenge
Dismissal of “PNET, primitive
neuroectodermal tumor“
-> “Embryonal tumor“
How many medulloblastoma groups are
(currently) defined based on genetics?
34
• A1. 3
• A2. 4
• A3. 5
• A4. 9
A2 is correct
Medulloblastoma
35
Long established histological
groups
Four genetically defined groups
Northcott et al, Nature Reviews 2012
Medulloblastoma
36
Surrogate IHC markers
for pathway activation
+ TP53 sequencing
+ MYC amplification by FISH
Genetics & histology & clinical factors stratify patients into risk groups
Louis DN et al, Acta Neuropathol 2016