In this report, we explore
retinoblastoma diagnostic accuracy and review
chemotherapy alternatives for
retinoblastoma using intravenous, intra-arterial, periocular, and intravitreal routes. A review of 2775 patients referred for management of
retinoblastoma, disclosed 78% with confirmed
retinoblastoma and 22% with simulating lesions, termed pseudoretinoblastomas. Children ≤2 years old showed leading pseudoretinoblastomas of persistent fetal vasculature,
Coats disease, and vitreous haemorrhage, whereas those >5 years showed simulators of Coats,
toxocariasis, and
familial exudative vitreoretinopathy. The diagnosis of
retinoblastoma should be established before planning therapeutic strategy.
Chemotherapy strategy depends on tumour laterality and stage of disease. If bilateral
retinoblastoma, intravenous
chemotherapy (IVC) is important as first-line
therapy for control of intraocular disease, prevention of
metastasis, and reduction in prevalence of pinealoblastoma and long-term second
malignant neoplasms. Bilateral groups D and E
retinoblastoma receive additional subtenon's
carboplatin boost for improved local control. If unilateral disease is present, then intra-arterial
chemotherapy (IAC) is often considered. IAC can be
salvage therapy following chemoreduction failure. Unilateral
retinoblastoma of groups D and E are managed with enucleation or globe-conserving IVC and/or IAC. Intravitreal
chemotherapy is cautiously reserved for recurrent vitreous seeds following other
therapies. In conclusion, the strategy for
retinoblastoma management with
chemotherapy depends on tumour laterality and stage of disease. Bilateral
retinoblastoma is most often managed with IVC and unilateral
retinoblastoma with IAC, but if advanced stage, combination IVC plus IAC or enucleation.