International Classification of Retinoblastoma Group Defining Features Group A Small tumors away from foveola and disc Tumors < 3 mm and Located at least 3 mm from foveola and 1.5 mm from the optic disc Group B All remaining tumors confined to the retina All other tumors confined to retina not in Group A Subretinal fluid < 3 mm from the base of tumor Group C Local subretinal fluid or seeding Local subretinal fluid alone > 3 to < 6 mm from tumor Vitreous seeding or subretinal seeding < 3 mm from the tumor Group D Diffuse subretinal fluid or seeding Subretinal fluid alone > 6 mm from tumor Vitreous seeding or subretinal seeding > 3 mm from tumor Group E Presence of poor prognosis features More than 2/3 globe filled with tumor Tumor in anterior segment or ciliary body Iris neovascularization, neovascular glaucoma Tumor necrosis, phthisis bulbi

Group A Small tumor (≤ 3 mm) confined to the retina, distant from the foveola and optic nerve.

Tumor > 3 mm, confined to the retina Group B Tumor > 3 mm, confined to the retina

Small tumors (≤ 3mm), confined to the retina, Group B Small tumors (≤ 3mm), confined to the retina, but in close proximity to optic nerve and foveola.

Tumor with localized subretinal fluid and local seeding Group C Tumor with localized subretinal fluid and local seeding

Tumor with diffuse seeding Group D Tumor with diffuse seeding

Tumor with diffuse subretinal fluid and subretinal seeding Group D Tumor with diffuse subretinal fluid and subretinal seeding

Tumor in anterior segment Group E Tumor in anterior segment

Recommended Treatment of RB R-E Group ABC Group Focal Treatments Chemotherapy Radiation I – II A + - If PD I – III B VCR 0.05 mg/kg CBP 18.6 mg/kg X 2 – 6 courses IV – V C – D +/- subtenon CBP x 3 CBP 14 mg/kg x 2 ETO 6 mg/kg x 2 X 6 courses If massive seeding, consider early RT Vb E Enucleation