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To maximize the preservation of useful vision, treatment should be undertaken in specialized centers, where there is collaboration between pediatric oncology and pediatric ophthalmology. The initial therapy for retinoblastoma is dependent on both

Table 23-3. International Classification System for Intraocular Retinoblastoma

GROUP A: Small intraretinal tumors away from foveola and disc GROUP B: All remaining discrete tumors confined to the retina GROUP C: Discrete local disease with minimal subretinal or vitreous seeding GROUP D: Diffuse disease with significant vitreous or subretinal seeding GROUP E: Presence of any one or more of these poor prognostic features: Tumor touching the lens

Tumor anterior to the 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 the intraocular and extraocular extent of the disease. Therapeutic modalities include the following and a combined modality approach is not uncommon:

1. Systemic chemotherapy

2. External beam radiotherapy

3. Local ophthalmic therapy, including local administration of chemotherapy

4. Enucleation.

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