Targeted Retinoblastoma Management
Targeted Retinoblastoma Management
The diagnosis of retinoblastoma is usually based on characteristic clinical features. Unlike most other cancers, the diagnosis is established clinically and without a biopsy. The characteristic features depend on the stage of disease. Early retinoblastoma appears as a translucent gray-white intraretinal mass with slightly dilated feeding and draining blood vessels. This tumor can be clinically detected when as tiny as 100 μm using indirect ophthalmoscopy. As retinoblastoma enlarges, a more opaque white appearance with prominent intrinsic vasculature is seen, and the tumor can produce surrounding subretinal fluid, subretinal seeds, and overlying vitreous seeds. With continued growth, retinoblastoma can produce total retinal detachment up against the lens, angle closure glaucoma, iris neovascularization, and can invade into surrounding structures such as the optic nerve, choroid, sclera, and orbit.
There are several conditions that can simulate retinoblastoma. In a review of 2775 patients referred for management of retinoblastoma to an ocular oncology center, there were 78% with clinically confirmed retinoblastoma and 22% with simulating lesions (pseudoretinoblastoma). The leading pseudoretinoblastoma varied slightly depending on patient's age. The retinoblastoma simulators in children aged 2 years or younger most often included persistent fetal vasculature (persistent hyperplastic primary vitreous), Coats disease, and vitreous hemorrhage, compared with children aged 5 years or older who showed simulators of Coats disease, toxocariasis, and familial exudative vitreoretinopathy (Table 1). The diagnosis of retinoblastoma should be unequivocally established before planning chemotherapeutic strategy.
The Clinical Features and Leading Simulators of Retinoblastoma
The diagnosis of retinoblastoma is usually based on characteristic clinical features. Unlike most other cancers, the diagnosis is established clinically and without a biopsy. The characteristic features depend on the stage of disease. Early retinoblastoma appears as a translucent gray-white intraretinal mass with slightly dilated feeding and draining blood vessels. This tumor can be clinically detected when as tiny as 100 μm using indirect ophthalmoscopy. As retinoblastoma enlarges, a more opaque white appearance with prominent intrinsic vasculature is seen, and the tumor can produce surrounding subretinal fluid, subretinal seeds, and overlying vitreous seeds. With continued growth, retinoblastoma can produce total retinal detachment up against the lens, angle closure glaucoma, iris neovascularization, and can invade into surrounding structures such as the optic nerve, choroid, sclera, and orbit.
There are several conditions that can simulate retinoblastoma. In a review of 2775 patients referred for management of retinoblastoma to an ocular oncology center, there were 78% with clinically confirmed retinoblastoma and 22% with simulating lesions (pseudoretinoblastoma). The leading pseudoretinoblastoma varied slightly depending on patient's age. The retinoblastoma simulators in children aged 2 years or younger most often included persistent fetal vasculature (persistent hyperplastic primary vitreous), Coats disease, and vitreous hemorrhage, compared with children aged 5 years or older who showed simulators of Coats disease, toxocariasis, and familial exudative vitreoretinopathy (Table 1). The diagnosis of retinoblastoma should be unequivocally established before planning chemotherapeutic strategy.
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