Clinical spectrum and treatment outcome of retinoblastoma with Group D and E diseases

Abstract

The purpose of our study was to evaluate the ocular survival and event-free survival after multimodal therapy for group D and E of retinoblastoma (RB). Enucleation of group D and E is controversial as the risks of chemotherapy must be weighed against the potential for vision. A 10-year retrospective study from one center of 86 patients with advanced intraocular disease defined as International Classification Retinoblastoma (ICRB) group “D” or “E.” Cases with visible extraocular extension at diagnosis were excluded. Ocular survival and patient survival were assessed. Indirect ophthalmoscopy at examination under anesthesia to visualize the tumor was used to evaluate clinical response. The median onset age in 86 patients with group D or E eye was 16 months (1–167 months). There were 29 (34%) bilateral cases. Leukocoria was the most common presentation sign (61%). Chemoreduction was primarily used in the treatment of intraocular RB. Selective ophthalmic arterial injection (SOAI) was applied as a component of multimodal therapy in 34 of the 86 cases. The globe preservation rate in patients with group D or E eyes was 19%. Using chemoreduction for advanced eyes, more eyes are being preserved which enables 70% 5-year ocular survival in patients with group D eyes. In triaging appropriate patients, multidisciplinary strategy can reduce tumor size with chemoreduction and consolidate the regressed tumor with local ophthalmic therapy to ensure globe salvage.