Bilateral advanced (group D or E) intraocular retinoblastoma: outcomes in 72 Asian Indian patients

Abstract

To study the clinical presentation, treatment, and outcomes of patients with bilateral advanced intraocular retinoblastoma. Retrospective case series of 72 patients. The mean age at presentation was 19 months. Leukocoria ( n  = 49, 68%) was the most common presenting complaint. The tumors were classified as groups D ( n  = 60, 42%) or E ( n  = 84, 58%) based on the Philadelphia version of International Classification of Retinoblastoma (ICRB); groups D ( n  = 84, 58%) or E ( n  = 60, 42%) based on Children’s Hospital Los Angeles version of International Classification of Intraocular Retinoblastoma (ICIoR); T2 ( n  = 116, 81%) or T3 ( n  = 28, 19%) based on 8th edition American Joint Committee Classification (AJCC). Systemic chemotherapy ( n  = 138, 96%) was the most common primary treatment modality. The chance of globe salvage was higher for group D based on ICRB (83%; odds ratio (OR) 7.73; 95% confidence interval (CI) 3.45–17.33) or ICIoR (81%; OR 12.75; 95% CI 5.74–28.34) and T2b (73%; OR 5.19; 95% CI 2.51–10.73) based on AJCC. Over a mean follow-up period of 59 months, tumor recurrence was noted in 42 (29%) eyes and globe salvage was achieved in 83 (58%) eyes. Of the 50 eyes where vision was recorded, vision of 20/200 or better was achieved in 24 (48%) eyes. There were events of leukemia ( n  = 1, 1%), pinealoblastoma ( n  = 1, 1%), systemic metastasis ( n  = 3, 4%), and death ( n  = 4, 6%) during the follow-up period. Multimodality treatment allows globe salvage (58%) and vision salvage (48%) in eyes with advanced group D and E intraocular retinoblastoma.