paediatrics Brussels 17

Medulloblastoma European Consensus 2015 • CONCENSUS Day 1: • All tumours subtyped by 450 K array or validated method - preferably 2 techniques as part of initial clinical workup • Neurosurgeons should aim for maximal safe removal: NTR (to be defined) is acceptable and prognostically equivalent to GTR for staging • QOL short, medium and long term is a high priority and should be evaluated in all patients • Reduced CSI RTX for STR/NTR + M0; re evaluate 1.5 cm 2 residual as high risk criterion • All wnt properly subtyped < 16 years old have excellent prognosis and should be treated with reduced radiation/chemotherapy • SHH + TP53 mutation = very poor prognosis: new treatment options needed especially if germline TP53 mutation • Every SHH patient/family should be offered genetic counselling • All SHH tumours should be sequenced for somatic and germline mutations of TP53, PTCH, SUFU as part of the diagnostic process • Recurrent tumours should be rebiopsied before using targeted therapy or 2 years beyond initial diagnosis or diagnosis is in doubt • Central review of MRI scans, pathology and radiotherapy planning in real time for considered for clinical trial or registry • All patients should be treated on a molecularly informed clinical trial. • Snap frozen tissue, paraffin embedded, blood and CSF should be collected on all patients