341 / 1708
Nr. 8
PNET5 – Stra8fica8on
Medulloblastoma ≥3-5years, <22 Years
(except WNT-HR, SHH-TP53 and Registry)
Cancer predisposi$on syndrome iden$fied
(except for SHH-TP53)
WNT-MB
PNET 5MB Registry
PNET 5 MB
SHH-TP53
(T Milde)
PNET 5
MB-LR
(F Doz)
PNET 5 MB
WNT-HR
(F Doz)
Group 3/4 and
SHH-
TP53
wt
SHH-
TP53mut
(soma$c or germline)
CTNNB1
pos. and
no HR features
No HR features
(Gr4/NMYC no longer HR)
Screening as by PNET5-MB Version 11 plus:
Subgrouping 2
nd
method mandatory
Tes8ng for germline
TP53, PTCH, SUFU
muta8ons in SHH-Medulloblastoma mandatory
Tes$ng recommended for
BRCA2
and
PALB2
in SHH-MB and
APC
in
CTNNB1
neg. WNT-MB
yes
no
yes
no
PNET 5
MB-SR
(S Rutkowski)