paediatrics Brussels 17

PNET5 – Stra8fica8on

Nr. 8

Medulloblastoma ≥3-5years, <22 Years (except WNT-HR, SHH-TP53 and Registry)

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

Cancer predisposi$on syndrome iden$fied (except for SHH-TP53)

yes

PNET 5MB Registry

no

WNT-MB

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)

no

yes

PNET 5 MB-LR (F Doz)

PNET 5 MB-SR (S Rutkowski)

PNET 5 MB SHH-TP53 (T Milde)

PNET 5 MB WNT-HR (F Doz)