Abstract
Purpose:
Advanced retinoblastoma can present with a spectrum of clinical findings that often leads to enucleation. However, a decision on adjuvant treatment is based on the presence of high risk features (HRF) determined on pathologic examination. The aim of this study is to determine whether certain clinical features of advanced disease could predict pathologic HRF that would merit adjuvant treatment.
Methods:
Medical records and histopathology of enucleated specimens from 69 patients who underwent enucleation prior to receiving any treatment at King Khaled Eye Specialist Hospital over were reviewed. Clinical findings were recorded in a database. The histologic slides were evaluated for HRF including anterior chamber angle invasion, iris invasion, ciliary body invasion, massive choroidal invasion, optic nerve invasion beyond lamina cribrosa, intrascleral infiltration, and extrascleral extension. Univariate analysis was performed to determine odd ratio
Results:
Sixty nine eyes (34 boys and 35 girls; mean age 19 months) were included in this study . Hyphema was associated with iris invasion [Odd’s ratio (OR) = 12)] and ciliary body invasion (OR = 12). Buphthalmos was associated with massive choroidal invasion (OR = 30.5), optic nerve invasion (OR = 10). Increased intraocular pressure was associated with anterior chamber angle invasion (OR = 3.9), ciliary body invasion (OR = 13)] and optic nerve invasion (OR = 9.5). Vitreous seeding was associated with anterior chamber angle invasion (OR = 3.3). Ectropion uvea was associated with ciliary body invasion (OR = 20.7), massive choroidal invasion (OR = 45.7) and optic nerve invasion (OR = 36). Massive choroidal invasion could be predicted by clinical signs of buphthalmos [Adj OR = 0.08 (95% CI 0.007 - 0.9)] and uveal ectropion [Adj OR = 0.05 (95% CI 0.004 - 0.7 )].
Conclusions:
This study suggests that certain clinical features in advanced retinoblastoma can be associated with HRF on pathologic examination. The presence of buphthalmos and uveal ectropion at presentation correlated with at least 2 HRF massive choroidal invasion ..and ciliary body invasion.
Keywords: 703 retinoblastoma •
638 pathology: human