Cilengitide in Combination With Irradiation in Children With Diffuse Intrinsic Pontine Glioma

Inclusion Criteria:

* Histologically confirmed diffuse intrinsic pontine glioma
* Metastatic disease allowed
* MRI measurable disease according to the WHO criteria and for extension cohort

  * Patient is able to undergo functional MRI (diffusion, perfusion, spectro)
  * Patient is able to undergo FDG-PET and sestamibi SPECT
* Life expectancy \> 8 weeks after the start of study treatment.
* No prior chemotherapy for the present cancer; no treatment for any other cancer during the last 5 years.
* No prior cerebral radiation therapy
* Age \> 6 months and \< 21 years
* Lansky Play Scale \> 50 or ECOG Performance Status \< 2; NB: Children and young adults with a worse performance status due to glioma-related motor paresis can be included.
* Absolute neutrophils count \> 1.5 x 109/l, Platelets \> 100 x 109/l
* Total bilirubin \< 1,5 x ULN, AST and ALT\< 2,5 x ULN
* Serum creatinine ≤ 1,5 X ULN for age. If serum creatinine \> 1,5 ULN, creatinine clearance must be \> 70 ml/min/1.73 m² (EDTA radioisotope GFR or 24 hours urines collection)
* Normal coagulation tests : prothrombin rate (prothrombin time = PT), TCA (PTT), fibrinogen
* No current organ toxicity \> grade 2 according to the NCICTCAE version 4.0, especially cardiovascular or renal disease (nephrotic syndrome, glomerulopathy, uncontrolled high blood pressure despite adequate treatment). In case of known or possible cardiac disease, a cardiological advice will be required prior to the inclusion in the study
* If anticonvulsants are currently administered, the dosing regimen must be stable within 1 week prior to the first dose of Cilengitide
* If corticosteroids are administered, the dosing regimen must be stable ≥ 5 days prior to the first dose of Cilengitide.
* Effective contraception for patients (male and female) of reproductive potential during their entire participation in the study and during 6 months after the last administration of Cilengitide.
* Negative pregnancy test (serum beta-HCG) within 1 week prior to start of study treatment in females of reproductive potential
* Patient covered by government health insurance
* Written informed consent given by patient and/or parents/ guardians prior to the study participation

Exclusion Criteria:

* Inclusion criteria failure
* History of coagulation disorder associated with bleeding or recurrent thrombotic events.
* Prior anti-angiogenic therapy
* Any other concomitant anti-cancer treatment not foreseen by this protocol.
* Concomitant inclusion in another therapeutic clinical trial; participation in another therapeutic clinical trial during the last 30 days.
* Pregnancy or breast feeding woman
* Uncontrolled intercurrent illness or active infection
* Unable for medical follow-up (geographic, social or mental reasons)