conservative treatments of retinoblastoma

Conditions: Retinoblastoma; Children; Retinal Neoplasm

Summary: conservative treatments of retinoblastoma (retino 2011) 1. -multicentric non randomised, phase ii study for the patients treated by chemoreduction (vp16, carboplatin) followed by chemothermotherapy without laser treatment at day 8 2. -multicentric non randomised, phase ii study for the patients with bilateral very asymmetric dis-ease (group d eye on one of the eye) or unilateral presentation groups b/c/d according to the age and vitreous seeding 3. - multicentric non randomised, phase ii study for the patients treated by 6 cycles of three drugs regimen and local treatments for bilateral group d eyes or on the only eye.


Eligibility criteria:
inclusion criteria: study 1 inclusion criteria: * patients affected by unilateral retinoblastoma groups a, b (according to the age), group c (according to the age and vitreous seeding),or bilateral retinoblastoma groups a, b, c (excluding the eyes with macular threat and bilateral group d eyes or on the only remaining eye) amenable to a conservative treatment (at least on one eye in bilateral disease) but needing initial chemotherapy because of the location, the size of the lesion (more than 4 mm of diameter), a vision threat or risks of intravitreal relapse making those patients not amenable to chemothermotherapy first line. * patients less than six months of age with unilateral retinoblastoma groups b, c, d, or bilateral very asymmetric with one eye group d and the other amenable to local treatments without chemotherapy. * children from 0 to 6 years old. study 2 inclusion criteria: * patients affected by unilateral or bilateral retinoblastoma group b (according to the age), group c (according to the age and vitreous seeding), or group d. * patients affected by bilateral retinoblastoma very asymmetric with a group d eye that can be treated by intraarterial chemotherapy by melphalan and the other amenable to local treatments without chemotherapy. * children from 6 months to 6 years old. study 3 inclusion criteria: * children affected of bilateral group d retinoblastoma or on the only eye amenable to conservative treatment. * children from 0 to 6 years old. common inclusion criteria: * patients not previously treated by chemotherapy or radiotherapy for this tumour or another cancer. * no contra-indications to the study treatments * possible long term follow-up. * written informed consent of the parents or the legal representative. * patients having social security cover. exclusion criteria: study 1 exclusion criteria: * patients for whom a local treatment is possible without initial chemotherapy (tumour smaller than 4 mm and located far from optic nerve head or macula). * patients with an unilateral group d with massive tumour or group e eyes needing enucleation first line or after initial chemotherapy (in case of buphthalmia or suspected optic nerve invasion or extrascleral extension). * patients affected by bilateral retinoblastoma very asymmetric with a group d eye that can be treated by intraarterial chemotherapy by melphalan and the other amenable to local treatments without chemotherapy. * patients with bilateral retinoblastoma and bilateral group d eyes or on the only remaining eye or presenting a bilateral macular threat requiring conservative treatment by a 6 cycles, three drugs regimen. study 2 exclusion criteria: * patients with a unilateral group d (extensive) or b or c but covering the optic nerve head or group e eyes for which enucleation is warranted first line or after chemotherapy (in case of buphthalmia or suspected optic nerve invasion or extrascleral extension). * patients with unilateral group d eye with tumour volume of more than 50% of eye volume, for whom a massive choroidal invasion could be associated (on clinical or imaging criteria) and for which enucleation is warranted. study 3 exclusion criteria: * patients for whom a local treatment is possible without chemoreduction (tumour smaller than 4 mm, distant from macula and from optic nerve head). * patients affected by bilateral retinoblastoma very asymmetric with a group d eye that can be treated by intraarterial chemotherapy by melphalan and the other amenable to local treatments without chemotherapy. * patients with bilateral retinoblastoma without macular threat or groups a, b, c than can be treated with chemoreduction by vp16 and carboplatin then chemothermotherapy without laser at day 8. common exclusion criteria: * patients older than 6 years old. * patients with extraocular retinoblastoma. * patients with a disease being a contra-indication to chemotherapy. * patients anteriorly treated by chemotherapy. * patients anteriorly treated by external beam irradiation. * patients anteriorly treated for another cancer. * follow-up not possible due to geographic distance from the center or for social or psychological reasons. * parents not having accepted the therapeutic strategy after explanations by the investigator. * contra-indication to the use of one of the drugs used in the study.