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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