Adjuvant Treatment in Extensive Unilateral Retinoblastoma Primary Enucleated (RB SFCE 2009)

Registration Number
NCT02870907
Lead Sponsor
Institut Curie
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 .
...

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
185
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.

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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

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Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
High risk groupOrbital irradiation* Orbital irradiation * 3 cycles of two different types of alternating chemotherapy courses (id 6 courses) : * Etoposide (100 mg/m²/d) and Carboplatin (160 mg/m²/d) with intrathecal Thiotepa injection. * Vincristin (1,5 mg/m²/d) - Cyclophosphamide (1000 mg/m²/d) * Cytapheresis for peripheral blood stem cells collection after the primary or the secondary courses of Vincristine- Cyclophosphamide. * High dose chemotherapy : * Carboplatin (AUC : 7/d) - etoposide (250 mg/m²/d) - Thiotepa (300 mg/m²/d) * Peripheral bood stem cell transplantation.
Low risk groupObservationNo treatment
High risk groupCytapheresis* Orbital irradiation * 3 cycles of two different types of alternating chemotherapy courses (id 6 courses) : * Etoposide (100 mg/m²/d) and Carboplatin (160 mg/m²/d) with intrathecal Thiotepa injection. * Vincristin (1,5 mg/m²/d) - Cyclophosphamide (1000 mg/m²/d) * Cytapheresis for peripheral blood stem cells collection after the primary or the secondary courses of Vincristine- Cyclophosphamide. * High dose chemotherapy : * Carboplatin (AUC : 7/d) - etoposide (250 mg/m²/d) - Thiotepa (300 mg/m²/d) * Peripheral bood stem cell transplantation.
High risk groupPeripheral bood stem cell transplantation* Orbital irradiation * 3 cycles of two different types of alternating chemotherapy courses (id 6 courses) : * Etoposide (100 mg/m²/d) and Carboplatin (160 mg/m²/d) with intrathecal Thiotepa injection. * Vincristin (1,5 mg/m²/d) - Cyclophosphamide (1000 mg/m²/d) * Cytapheresis for peripheral blood stem cells collection after the primary or the secondary courses of Vincristine- Cyclophosphamide. * High dose chemotherapy : * Carboplatin (AUC : 7/d) - etoposide (250 mg/m²/d) - Thiotepa (300 mg/m²/d) * Peripheral bood stem cell transplantation.
Intermediate risk sub group 1Cyclophosphamide2 cycles (4 courses): 2 courses of etoposide and Carboplatin from D1 to D5 and Vincristin at D22 and D26- Cyclophosphamide from D22 to D26.
Intermediate risk sub group 1Etoposide2 cycles (4 courses): 2 courses of etoposide and Carboplatin from D1 to D5 and Vincristin at D22 and D26- Cyclophosphamide from D22 to D26.
Intermediate risk sub group 2Vincristine2 courses of Vincristin and Carboplatin
Intermediate risk sub group 1Carboplatin2 cycles (4 courses): 2 courses of etoposide and Carboplatin from D1 to D5 and Vincristin at D22 and D26- Cyclophosphamide from D22 to D26.
Intermediate risk sub group 1Vincristine2 cycles (4 courses): 2 courses of etoposide and Carboplatin from D1 to D5 and Vincristin at D22 and D26- Cyclophosphamide from D22 to D26.
Intermediate risk sub group 2Carboplatin2 courses of Vincristin and Carboplatin
High risk groupCarboplatin* Orbital irradiation * 3 cycles of two different types of alternating chemotherapy courses (id 6 courses) : * Etoposide (100 mg/m²/d) and Carboplatin (160 mg/m²/d) with intrathecal Thiotepa injection. * Vincristin (1,5 mg/m²/d) - Cyclophosphamide (1000 mg/m²/d) * Cytapheresis for peripheral blood stem cells collection after the primary or the secondary courses of Vincristine- Cyclophosphamide. * High dose chemotherapy : * Carboplatin (AUC : 7/d) - etoposide (250 mg/m²/d) - Thiotepa (300 mg/m²/d) * Peripheral bood stem cell transplantation.
High risk groupEtoposide* Orbital irradiation * 3 cycles of two different types of alternating chemotherapy courses (id 6 courses) : * Etoposide (100 mg/m²/d) and Carboplatin (160 mg/m²/d) with intrathecal Thiotepa injection. * Vincristin (1,5 mg/m²/d) - Cyclophosphamide (1000 mg/m²/d) * Cytapheresis for peripheral blood stem cells collection after the primary or the secondary courses of Vincristine- Cyclophosphamide. * High dose chemotherapy : * Carboplatin (AUC : 7/d) - etoposide (250 mg/m²/d) - Thiotepa (300 mg/m²/d) * Peripheral bood stem cell transplantation.
High risk groupThiotepa* Orbital irradiation * 3 cycles of two different types of alternating chemotherapy courses (id 6 courses) : * Etoposide (100 mg/m²/d) and Carboplatin (160 mg/m²/d) with intrathecal Thiotepa injection. * Vincristin (1,5 mg/m²/d) - Cyclophosphamide (1000 mg/m²/d) * Cytapheresis for peripheral blood stem cells collection after the primary or the secondary courses of Vincristine- Cyclophosphamide. * High dose chemotherapy : * Carboplatin (AUC : 7/d) - etoposide (250 mg/m²/d) - Thiotepa (300 mg/m²/d) * Peripheral bood stem cell transplantation.
High risk groupVincristine* Orbital irradiation * 3 cycles of two different types of alternating chemotherapy courses (id 6 courses) : * Etoposide (100 mg/m²/d) and Carboplatin (160 mg/m²/d) with intrathecal Thiotepa injection. * Vincristin (1,5 mg/m²/d) - Cyclophosphamide (1000 mg/m²/d) * Cytapheresis for peripheral blood stem cells collection after the primary or the secondary courses of Vincristine- Cyclophosphamide. * High dose chemotherapy : * Carboplatin (AUC : 7/d) - etoposide (250 mg/m²/d) - Thiotepa (300 mg/m²/d) * Peripheral bood stem cell transplantation.
High risk groupCyclophosphamide* Orbital irradiation * 3 cycles of two different types of alternating chemotherapy courses (id 6 courses) : * Etoposide (100 mg/m²/d) and Carboplatin (160 mg/m²/d) with intrathecal Thiotepa injection. * Vincristin (1,5 mg/m²/d) - Cyclophosphamide (1000 mg/m²/d) * Cytapheresis for peripheral blood stem cells collection after the primary or the secondary courses of Vincristine- Cyclophosphamide. * High dose chemotherapy : * Carboplatin (AUC : 7/d) - etoposide (250 mg/m²/d) - Thiotepa (300 mg/m²/d) * Peripheral bood stem cell transplantation.
Primary Outcome Measures
NameTimeMethod
Rate of extra ocular relapses5 years
Secondary Outcome Measures
NameTimeMethod
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 bilateralisation5 years
Evaluate the different histopathological risk factors frequency5 years

Number of patient in each histopathological risk group

To determine tumors genomicat 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 extensionAt the inclusion

Number of extra ocular extension detected by MRI

Trial Locations

Locations (27)

Chr Felix Guyon

🇫🇷

Saint-Denis, La Réunion, France

Hopital Nord Chu Amiens

🇫🇷

Amiens, France

Chu Angers

🇫🇷

Angers, France

Hopital Jean Minioz

🇫🇷

Besancon, France

Chu R; Pellegrin

🇫🇷

Bordeaux, France

Chu Morvan

🇫🇷

Brest, France

Centre Oscar Lambret

🇫🇷

Lille, France

Institut Curie

🇫🇷

Paris, France

Hopital D'Enfants La Timone

🇫🇷

Marseille, France

Chur de Reims

🇫🇷

Reims, France

Chu Toulouse

🇫🇷

Toulouse, France

Chu Nancy

🇫🇷

Vandoeuvre Les Nancy, France

CHU CAEN

🇫🇷

Caen, France

Chu Estaing

🇫🇷

Clermont Ferrand, France

Chu Bocage

🇫🇷

Dijon, France

Chu de Grenoble

🇫🇷

Grenoble, France

Centre Leon Berard

🇫🇷

Lyon, France

Hopital Arnaud de Villeneuve

🇫🇷

Montpellier, France

Chu Nantes

🇫🇷

Nantes, France

Chu de Poitiers

🇫🇷

Poitiers, France

Chu de Rouen

🇫🇷

Rouen, France

Hoptial Hautepierre

🇫🇷

Strasbourg, France

Chu Tours

🇫🇷

Tours, France

Chu de Nice

🇫🇷

Nice, France

Chu Limoges

🇫🇷

Limoges, France

Chu de Rennes

🇫🇷

Rennes, France

Chu Saint Etienne

🇫🇷

Saint Etienne, France

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