Clinical trial NCT02870907: Adjuvant Treatment in Extensive Unilateral Retinoblastoma Primary Enucleated (RB SFCE 2009)
Official title: Adjuvant Treatment in Extensive Unilateral Retinoblastoma Primary Enucleated
Study type: INTERVENTIONAL
Overall status: RECRUITING
Phase: PHASE2
Conditions: Retinoblastoma
Keywords: primary enucleation
retinoblastoma
Lead sponsor: Institut Curie
Sponsor class: OTHER
First posted: 2016-08-17
Enrollment: 185
Sex: ALL
Minimum age (years): 2
Maximum age (years): 10
Standard ages: CHILD
Accepts healthy volunteers: no
Allocation: NON_RANDOMIZED
Intervention model: PARALLEL
Primary purpose: TREATMENT

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 .

Eligibility criteria: 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

Primary outcomes:
measure: Rate of extra ocular relapses
timeFrame: 5 years
Secondary outcomes:
measure: Evaluate long term and acute toxicities of adjuvant chemotherapy and orbital irradiation if necessary.
timeFrame: 5 years
measure: Number of patient with secondary bilateralisation
timeFrame: 5 years
measure: Evaluate the different histopathological risk factors frequency
timeFrame: 5 years
measure: To determine tumors genomic
timeFrame: at the inclusion
measure: Evaluate sensitivity of MRI in detecting extra ocular extension
timeFrame: At the inclusion
Other outcomes:
Locations:
city: Saint-Denis
country: France
facility: Chr Felix Guyon
state: La Réunion
city: Amiens
country: France
facility: Hopital Nord Chu Amiens
city: Angers
country: France
facility: Chu Angers
city: Besancon
country: France
facility: Hopital Jean Minioz
city: Bordeaux
country: France
facility: Chu R; Pellegrin
city: Brest
country: France
facility: Chu Morvan
city: Caen
country: France
facility: CHU CAEN
city: Clermont Ferrand
country: France
facility: Chu Estaing
city: Dijon
country: France
facility: Chu Bocage
city: Grenoble
country: France
facility: Chu de Grenoble
city: Lille
country: France
facility: Centre Oscar Lambret
city: Limoges
country: France
facility: Chu Limoges
city: Lyon
country: France
facility: Centre Leon Berard
city: Marseille
country: France
facility: Hopital D'Enfants La Timone
city: Montpellier
country: France
facility: Hopital Arnaud de Villeneuve
city: Nantes
country: France
facility: Chu Nantes
city: Nice
country: France
facility: Chu de Nice
city: Paris
country: France
facility: Institut Curie
city: Poitiers
country: France
facility: Chu de Poitiers
city: Reims
country: France
facility: Chur de Reims
city: Rennes
country: France
facility: Chu de Rennes
city: Rouen
country: France
facility: Chu de Rouen
city: Saint Etienne
country: France
facility: Chu Saint Etienne
city: Strasbourg
country: France
facility: Hoptial Hautepierre
city: Toulouse
country: France
facility: Chu Toulouse
city: Tours
country: France
facility: Chu Tours
city: Vandoeuvre Les Nancy
country: France
facility: Chu Nancy
Study officials:
References: