Neoadjuvant Carboplatin and Vincristine and Standard Local Ophthalmic Therapy in Treating Patients With Intraocular Retinoblastoma

Inclusion Criteria:

• Newly diagnosed Group B intraocular retinoblastoma meeting 1 of the following criteria:

• Group B tumor(s) in 1 eye

• Group B tumor(s) in both eyes

• Group A tumor in 1 eye and Group B tumor(s) in the other eye

• Group E tumor in 1 eye that has been enucleated and Group B tumor(s) in the remaining eye at the time of enucleation of the Group E tumor

• Defined by the International Classification System for Intraocular Retinoblastoma as follows:

• Group A: Small tumors (≤ 3 mm in greatest dimension) confined to the retina, away from foveola and disc meeting the following criteria:

• More than 3 mm from fovea

• More than 1.5 mm from optic disk

• Group B: Tumors more than 3 mm meeting the following criteria:

• Confined to the retina in any location not in Group A

• Tumor associated subretinal fluid < 3 mm from the tumor margin with no subretinal seeding

• Group E: Must have ≥ 1 of the following present:

• Tumor touching the lens

• Tumor anterior to anterior vitreous face involving ciliary body or anterior segment

• Diffuse infiltrating retinoblastoma

• Neovascular glaucoma

• Opaque media from hemorrhage

• Tumor necrosis with aseptic orbital cellulites

• Phthisis bulbi

• Confirmation of diagnosis by CT scan or MRI of the brain and orbits AND an ophthalmologic evaluation under anesthesia within the past 3 weeks

• No choroidal and/or optic nerve invasion past the lamina cribosa

• No evidence of extraocular retinoblastoma clinically or by head and orbital MRI and/or CT scan

• No tumor present on histological exam at the cut end of the optic nerve for any Group E eye enucleated before study entry

• Performance status - ECOG 0-2

• Bilirubin ≤ 1.5 times upper limit of normal (ULN) for age

• AST or ALT < 2.5 times ULN for age

• Creatinine clearance (based on Schwartz formula) or radioisotope glomerular filtration rate ≥ 70mL/min/1.73 m^2

• No prior chemotherapy

• No other concurrent chemotherapy

• No prior radiotherapy

• No other concurrent radiotherapy, including intensity-modulated stereotactic, or proton beam radiotherapy

• Prior enucleation of one eye allowed provided the remaining eye is Group B

• No concurrent enucleation

• No prior local ophthalmic therapy for retinoblastoma

• No other prior therapy for retinoblastoma

• No local therapy during chemotherapy course 1