Protocol for the Study and Treatment of Participants With Intraocular Retinoblastoma

Given via minibag/gravity flow.

Given IV over 30 minutes.

Given subcutaneously 24-36 hours after chemotherapy for 7-10 days, until absolute neutrophil count (ANC) is \>2,000/µL on one occasion after the expected nadir.

Given subcutaneously 24-36 hours after chemotherapy for 7-10 days, until ANC is \>2,000/µL on one occasion after the expected nadir.

Given IV over 60 minutes. Given periocular (subtenon/subconjunctival).

Focal treatments will be administered at the discretion of the treating team to strata A, B and D. In select cases of very early stage retinoblastoma (Stratum A), participants may receive focal therapies only and chemotherapy will be held at the discretion of the treating team. If there is any evidence of progression or unsatisfactory results, the participant will begin chemotherapy as per Stratum A. For selected participants an effort will be made to perform sequential chemo-thermotherapy. In these cases, carboplatin will be administered one or two hours prior to thermotherapy.

Given IV. Participants who cannot tolerate etoposide may be given etoposide phosphate (Etopophos(R)).

Given IV.

Given IV before CYCLO and at 3, 6 and 9 hours after CYCLO.

Given IV on Day 1 of Cycles 2, 4 and 6 in Stratum C high-risk.

Eye removal due to advanced disease in Strata C and D participants.

EBRT or proton beam radiation will be administered to any eye in which the disease is considered to be not controllable with focal treatments alone, and in participants with enucleated eyes in which high risk of orbital and/or central nervous system disease is documented histologically (high-risk group with disease extension beyond the sclera or cornea, or beyond the cut end of the optic nerve). EBRT will be administered using standard techniques practices with the objective of limiting dose to normal tissues including the hypothalamic-pituitary unit, supratentorial brain, orbit, cochleae and contralateral eye when indicated. Participants will be evaluated on an individual basis to determine whether they might benefit from referral for proton therapy.