Treatment of High-Risk Cerebral Primitive Neuroectodermal Tumors in Children Aged Over 5 Years

Inclusion criteria

• Type of tumor:

• Metastatic medulloblastoma whatever the quality of the initial resection (radiologically visible metastases by MRI and/or CSF invaded by at least one mass of tumoral cells).
• Incompletely resected local medulloblastoma with a residue > 1.5 cm2.
• Anaplastic, large cell medulloblastoma whatever the risk criteria (localized or metastatic, complete or incomplete resection).
• Medulloblastoma with amplification of c-myc or N-myc whatever the risk criteria (local or metastatic, complete or incomplete resection).
• Local and/or metastatic sustentorial PNET.
• Age at diagnosis of the medulloblastoma of more than 5 years and less than 20 years.
• Age at diagnosis of S-PNET of more than 10 years and less than 20 years.
• Nutritional and general status compatible with treatment, Lansky score > 60.
• Estimated life expectancy > 1 months.
• Radiographs must be available for the second reading in dicom format on a CD-ROM.
• Hematological function at diagnosis: PMN > 1.0 x 109/l and platelets > 100 x 109/l.
• Hepatic function at diagnosis: serum bilirubin < 1.5 times normal value; ASAT and ALAT < 2.5 times normal values; prothrombin time > 50%; fibrinogen > 1.5 g/l.
• Renal function at diagnosis: serum creatinine according to age in a correctly hydrated child: 1 to 15 years < 65 micromol/l; 15 to 18 years < 110 micromol/l.
• No organ toxicity (Grade > 2 according to NCI-CTC coding, version 2.0)
• No other concomitant anti-cancer treatment.
• No prior anti-cancer therapy.
• No prior irradiation.
• Written informed consent signed by both parents or legal guardians

Exclusion criteria

• Failure to comply with one of the inclusion criteria.
• Severe or life-threatening infection.
• Uncontrolled active or symptomatic intracranial hypertension.
• Refusal of parents or legal guardian.
• Patients incapable of undergoing medical follow-up for geographical, social or mental reasons