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Biological Medicine for Diffuse Intrinsic Pontine Glioma (DIPG) Eradicatio

Phase 2
Recruiting
Conditions
diffuse intrinsic pontine glioma - brainstem glioma
10029211
Registration Number
NL-OMON49636
Lead Sponsor
Gustave Roussy
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
Recruiting
Sex
Not specified
Target Recruitment
15
Inclusion Criteria

*Diagnosis of DIPG (clinical and radiological, or histological in case the
biopsy was performed before study entry)
- Non-brainstem diffuse midline gliomas, H3K27M mutant (NB-DMG), will be
eligible for the trial after biopsy or surgery. As biopsy and surgery is
considered as standard practice for these locations, informed consent for the
biopsy will not be necessary. Patient will sign the consent after the diagnosis
to allow central review and biomarkers assessment thereafter.
*DIPG or NB-DMG at diagnosis: no prior chemotherapy for the present cancer; no
prior cerebral radiation therapy
*NB : Metastatic disease allowed. Patient with metastatic disease are eligible
for the study (including the randomised trial if diagnosis of DIPG/ NB-DMG
confirmed). In this situation, radiotherapy will have to start within three
weeks after the biopsy while targerted treatment will start at the end of the
irradiation.
*Age > 6 months and < 30 years. For children below the age of 3 years,
inclusion in the study and medical decisions should be discussed with the
coordinating investigator.
*Eligible for a biopsy, or biopsy performed for diagnostic purpose and material
available for the biomarker assessment
*Eligible for cerebral radiotherapy
*Patient covered by an health insurance if national requirement
*Written informed consent given by patient and/or parents/legal representative
for biomarkers assessment and registration in the study., *Eligibility criteria
for the study (see above)
*Confirmed histological diagnosis of diffuse intrinsic pontine glioma (grade
II, III, IV WHO), or NB-DMG confirmed by central pathology review (including
the assessment of the loss of H3K27me3 by immunohistochemistry or the presence
of a mutation in the histone H3 variant genes). Patients without classical
clinical and radiological diagnostic criteria who fulfil the histological and
biological criteria of DIPG are eligible for the trial. Pilocytic astrocytoma
and ganglioliomas are not eligible. Patients with a suspected DIPG but no
histological confirmation could be randomised if and only if the radiology is
typical of a DIPG as well as the short clinical history. This would need to be
reviewed and agreed centrally. Confirmation of the diagnosis of non-brainstem
diffuse midline gliomas, H3K27M mutant, by central review, is needed before the
randomisation of cases of NB-DMG
* PTEN-loss (evaluated by IHC). If the biomarker study is not contributive (ie
no information is obtained) but the diagnosis of DIPG is ascertained either by
a typical short clinical history and
typical radiological appearance or by the histology of diffuse glioma with
H3K27M trimethylation loss and eventually the detection of the H3K27M mutation
by immunohistochemistry, the patient will be included in the trial and will
receive everolimus.
*Life expectancy > 12 weeks after the start of study treatment
*Karnofsky performance status scale or Lansky Play Scale > 50%. The PS should
not take the neurologic deficit per se into account. NB: Children and young
adults with a worse performance status due to glioma-related motor paresis can
be included.
*Absolute neutrophil 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 >

Exclusion Criteria

Spontaneous massive intratumour bleeding. Patients with post-operative bleeding
will be allowed to enter the study provided the haemorrhage is controlled. Same
rule applies for the other post-operative complications (infection, CSF
leakage, absence of wound closure, subdural collection*).
*Any other concomitant anti-cancer treatment not foreseen by this protocol
*Any other cancer during the last 5 years
*Uncontrolled intercurrent illness or active infection
*Any other co-morbid condition that in the investigator*s opinion would impair
study participation
*Unable for medical follow-up (geographic, social or mental reasons)
*Patient not fulfilling one of the previous eligibility criteria.
*Patient previously treated with irradiation on the brainstem for another
neoplasm
*Patient with congenital galactose intolerance, Lapp lactase deficiency or
glucose-galactose malabsorption.
*Patient not covered by a social security agreement accepted in the treating
country if national requirement
*Pregnant or breast feeding women
*PTEN-positivity of the tumor cells (evaluated by IHC)

Study & Design

Study Type
Interventional
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
<p>The overall survival is the primary efficacy endpoint for the BIOMEDE Phase II<br /><br>trial.<br /><br>For the comparison to historical controls, the overall survival is computed<br /><br>from the date of biopsy (or initial surgery). The main analysis will be based<br /><br>on the whole survival curve.</p><br>
Secondary Outcome Measures
NameTimeMethod
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