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Nr. 11

PNET5 – SHH-TP53

Key inclusion criteria:

-

SHH MB with

TP53

muta$on

-

TP53

germline evalua$on mandatory

-

All histology subtypes

-

M0 and M+

-

>3-5 years with no upper age limit

MRI

MRI

MRI

MRI

MRI

MRI

LP

OP Chemotherapy Radiotherapy

Vinblas$n

≤4w 16w ≤4w 6w

4*-

6w

24w

* In pa$ents with local radiotherapy only

SKK like SKK like

+VCR

“SKK-like” chemotherapy (with i.ventr. MTX):

w1: Doxorubicin (or Carbopla$n)/VCR

w3, w5: HD-MTX/VCR

w7: Carbopla$n/VCR

Radiotherapy

(with weekly VCR 1,5mg/m

2

)

:

Germline

TP53mut

M0:

Focal RT

Safety margin 23.4 Gy, tumor 54.0 Gy,

Germline

TP53mut

M+:CSI 23.4 Gy

,

tumor 54.0 Gy

Soma$c

TP53mut

: CSI 36.0 Gy, tumor 54.0 Gy