NEOVASCULAR GLAUCOMA AS A PREDICTOR of RETINOBLASTOMA HIGH-RISK HISTOPATHOLOGY in AN INTERNATIONAL MULTICENTER STUDY.

Abstract

Purpose: To assess histopathology and outcomes after primary enucleation of eyes with retinoblastoma presenting with neovascular glaucoma (NVG). Methods: This was an international multicenter case series study across five continents. A retrospective review of patient charts was performed for all patients undergoing primary enucleation for retinoblastoma (n = 1,420) using a standardized data-collection spreadsheet. Clinical features, pathologic grade, and outcomes were compared between NVG patients and those with an American Joint Commission on Cancer eighth edition clinical stage of cT2. High-risk histopathology was defined as American Joint Commission on Cancer eighth edition pathologic stage = pT2b. Results: Neovascular glaucoma was seen in 224/1,420 (16%) patients. The mean age at presentation of those with NVG was 30 months (median 25, range 0-120 months), and 131 (58%) patients had high-risk histopathology. The univariate logistic regression odds ratio for NVG predicting high-risk histopathology was 1.73 (95% confidence interval: 1.3-2.31) and from multivariate logistic regression was 1.77 (95% confidence interval: 1.23-2.56). Patients with a longer duration of symptoms ( P = 0.03), buphthalmos ( P = 0.02), and ectropion uveae ( P < 0.01) were more likely to have high-risk histopathology. Patients with NVG were more likely to develop metastasis than cT2 patients ( P = 0.04). Conclusion: There is a significant association between NVG at presentation, high-risk histopathology, and metastatic risk.