Retinoblastoma
Conditions
Keywords
primary enucleation, retinoblastoma
Brief summary
Postoperative Treatment of Unilateral Retinoblastoma After Primary Enucleation according to histopathological risk factors of the International Retinoblastoma Staging Working Group.
Detailed description
Post operative chemotherapy +/- radiotherapy according to histopathological risk factors of the International Retinoblastoma Staging Working Group. * Low risk group : * No optic nerve involvement. * Intra and prelaminar involvement * No choroidal involvement. * Minimal superficial choroidal involvement . * Intermediate risk group, 2 sub groups : * Sub group 1 : * Retrolaminar involvement without Invasion of surgical margin associated or not to massive choroidal involvement * Anterior segment involvement. * Intrascleral involvement. * Sub Group 2 : * Isolated massive choroidal involvement. * High risk group : * Invasion of the surgical margin of the optic nerve * and/or microscopic extrascleral involvement * Optic nerve meningeal sheat involvement .
Interventions
OTHERObservation
no post operative chemotherapy
DRUGEtoposide
100 mg/m²/d, IV (in the vein) from D1 to D5.
DRUGVincristine
1, 5 mg/m²/d, IV at D1.
RADIATIONOrbital irradiation
45 Grays (Standard or external beam radiotherapy).
DRUGCarboplatin
160 mg/m²/d, IV from D1 to D5.
DRUGCyclophosphamide
300 mg/m²/d, IV from D22 to D26.
DRUGThiotepa
15 mg, intrathecal Thiotepa injection at D1.
PROCEDURECytapheresis
Cytapheresis for peripheral blood stem cells collection after the primary or the secondary courses of Vincristine- Cyclophosphamid.
PROCEDUREPeripheral bood stem cell transplantation
at D0
Sponsors
Institut Curie
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
2 Months to 10 Years
Healthy volunteers
No
Inclusion criteria
1. Written informed consent - a signed informed consent and/or assent (as age appropriate) will be obtained according to institutional guidelines; 2. Male or female ≥2 months and \<10 years of age at the time of signing the informed consent form; 3. Diagnosis of non familial extensive unilateral retinoblastoma treated by primary enucleation 4. In case of post operative chemotherapy, patients must have adequate organ function: * Adequate hematopoietic function Neutrophils\>1.0x109/l, Platelets \>100 x 109/l. * Adequate hepatic function: grade II NCI CTC * Adequate renal function: serum creatinemia \<1.5 x ULN for age with normal creatinine clearance estimated by SCHWARTZ formula * Audiometry \< Grade II de Brock. * Echocardiography normal in case of high dose cyclophosphamide chemotherapy (3 g/m²). 5. Patients affiliated to a Social Security Regimen or beneficiary of the same 6. No chemotherapy or radiotherapy prior to administration of the first dose of study treatment for retinoblastoma or other tumor types 7. Without medical cons-indication to study drugs.
Exclusion criteria
* Bilateral and/or familial or trilateral retinoblastoma. * Unilateral retinoblastoma with indication of primary chemotherapy before enucleation: * One or several surgical risk factors * Buphthalmia Exophthalmia. * Peri ocular inflammatory signs. * Extraocular extension : * Radiological retrolaminar extension (more than 3 mm behind the lamina cribrosa) and or meningeal sheat optic nerve extension. * Extrascleral extension * Lymp nodes extension * Unilateral retinoblastoma with possibility of conservative treatment: * Metastatic extension at diagnosis * One inclusion criteria non observed * Uncontrolled medical conditions, psychological, familial, sociological, or geographical conditions that do not permit compliance with the protocol
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Rate of extra ocular relapses | 5 years |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Evaluate long term and acute toxicities of adjuvant chemotherapy and orbital irradiation if necessary. | 5 years | Number of participants with treatment-related Adverse Events as assessed by CTCAE v3.0. |
| Number of patient with secondary bilateralisation | 5 years | — |
| Evaluate the different histopathological risk factors frequency | 5 years | Number of patient in each histopathological risk group |
| To determine tumors genomic | at the inclusion | Tumor genomic characterization in order to provide some new prognosis factors and better understanding of tumorigenesis by using of NGS (Next Generation Sequencing) techniques |
| Evaluate sensitivity of MRI in detecting extra ocular extension | At the inclusion | Number of extra ocular extension detected by MRI |
Countries
France
Outcome results
None listed