NEOVASCULAR GLAUCOMA AS A PREDICTOR OF RETINOBLASTOMA HIGH-RISK HISTOPATHOLOGY IN AN INTERNATIONAL MULTICENTER STUDY

To assess histopathology and outcomes after primary enucleation of eyes with retinoblastoma presenting with neovascular glaucoma (NVG).

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.

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).

There is a significant association between NVG at presentation, high-risk histopathology, and metastatic risk.