Treatment of metastatic disease in retinobastoma is
A i.e. Chemotherapy Treatment Plan of Retinoblastoma The factors influence the management include size, location & laterality of tumor, vision of affected & unaffected eye, age & health of child and any associated ocular problem such as retinal detachment, vitreous haemorrhage, neovascularization of iris & secondary glaucoma. Chemotherapy (CT) It is primary treatment option in children with bilateral retinoblastomaQ. Initial treatment in children with unilateral disease when the affected eye is believed to be salvageable. 6 cycles of Carboplatin, etopasaide & vincristine (CEV) +- cyclosporine is most common regimen. Paially regressed tumors that are still ble following the 2nd cycle of chemotherapy & any new tumors that develop during the course of chemotherapy must be treated by obliterative local therapies (cryotherapy, laser therapy, and episcleral plaque radiation therapy). Residual or recurrent vitreous seeds following chemotherapy and focal (local) treatments usually require external beam radiation therapy if eye is to be salvaged. Chemotherapy is also used to treat extraocular tumor extension at presentation, or detected on histopathology of enucleated eye, orbital tumor recurrence after enucleation, intracranial invasion by tumor & metastatic diseaseQ. Inspite of popularity of CT as the primary t/ t for RB, enucleation remains impoant option especially in - Unilateral advanced intraocular diseaseQ. - B/L for advanced disease not amenable to any eye preserving therapy. - For more severely affected eye in markedly asymmetrical bilateral cases. The principle route of exit of tumor cells from the eye is along the optic nerveQ Enucleation is usually curative in RB if an optic nerve section longer than 5mm is obtained with globe - Surgeon should attempt to obtain optic nerve section 10-15 mm long in every case. External Beam Radiation Therapy 40-50 Gy radiation (in multiple fractions of 150-200 cGy over 4-5 weeks) is given using a linear acclelerator to Eyes containing one or more tumors that involve optic disc. Eyes that show dffuse vitreous seeding. Eyes for which prior chemotherapy or local treatments such as laser therapy, cryotherapy, photocoagulation or plaque radiotherapy, failed. - Vitreous seeds generally do not respond well to , because of their relative hypoxic status. - Cataract (at least after 6 months usually after 1-1.5 years) is likely to develop. Rarely retinopathy, neovascular glaucoma, orbital bone growth arrest and second malignancy may develop.
Chemotherapy
Enucleation
Radiotherapy
Cryo