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)