An interventional, open-label, Phase II study, to evaluate safety and efficacy of standard and high-dose chemotherapy associated with craniospinal irradiation in patients with metastatic medulloblastoma and other embryonal tumours

Prior treatment

• Administration of a first-line chemotherapy cycle at the same time as the start of the study
• Concomitant partecipation to other clinical trial
• Concomitant administration of live attenuated vaccines

Other illness

• Any disease or condition that contraindicates the use of the study treatment (es. serious mental retardation, brain palsy, congenital syndrome, cardiomyopathy, psychosocial problems)

Other

• Other contraindications reported in the SmPC
• Pregnancy or breastfeeding
• Inadequate contraception by patients of both sexes
• Hypersensibility to active principles or excipients
• Severe bone-marrow depression
• Inability to comply for study follow-up

• First diagnosed, previously untreated, patients with high-risk medulloblastoma (according to WHO 2016 classification): metastatic; anaplastic or large cell; partially resected (residual volume on two floors> 1.5cm2) according to WHO classification (2016); N-MYC C-MYC amplified
• Written informed consent obtained from the patient/parents or legal representative
• Availability for treatment and ability to be compliant with the protocol
• Patients with other embryonic tumors (WHO classification 2016) - embryonic tumor with C19MC-altered multilayer rosettes; embryonic tumor with multilayer rosettes not otherwise specified, medulloepithelioma, CNS neuroblastoma, CNS ganglioneuroblastoma, CNS embryonic tumor not otherwise specified, CNS embryonic tumor with rhabdoid features - of first diagnosis, previously untreated (with chemo and radiotherapy) and treated only surgically
• First diagnosed Pinealoblastomas (WHO 2016), previously untreated (with chemo and radiotherapy) and treated only surgically
• Males and females aged >3 years and <21 years at time of the diagnosis
• Life expectancy > or = 12 months
• Karnofsky/Lansky > or = 40 %
• Adequate hematological function (leucocyte > or = 2.0 x 10^9/l - Hemoglobin > or = 10 g/dl - platelet > or = 50 x 10^9/l)
• Adequate liver function (total bilirubin < or = 2.5 x ULN - ALT/AST < or = 5.0 x ULN)
• Adequate renal function (serum creatinine < or = 1.5 x ULN)