Orbital Retinoblastoma: Enucleation vs. Ophthalmic Artery Chemosurgery for Advanced Intraocular Retinoblastoma

Abstract

Purpose: To determine the incidence of orbital recurrence following enucleation and ophthalmic artery chemosurgery (OAC) as primary treatments for advanced stage retinoblastoma.

Methods: Single center retrospective study of 73 patients (74 eyes) of Reese-Ellsworth group V, or International Classification of Retinoblastoma group D or E primarily treated with enucleation and 76 patients (90 eyes) primarily treated with OAC. Endpoints analyzed were the development of orbital disease, incidence of metastasis, and death from metastatic retinoblastoma.

Results: There were 6 orbital recurrences (incidence 8.1%) in the in the primary enucleation group and 1 orbital recurrence (incidence 1.1%) in the primary OAC group during median follow up times of 32.2 months (range 0.1-97.1) and 36.8 months (range 3.0-104.3) respectively. The 24-month Kaplan Meier estimate for orbital recurrence free survival was significantly worse for the enucleation group 92.6% (95% C.I. 83.2-96.8) than for the OAC group 98.5% (95% C.I. 89.9-99.7), Log-Rank p-value = 0.02. The enucleation group had 6 cases of metastatic disease and 2 deaths representing 8.2% and 2.7% of patients respectively. In the OAC group, there were 3 (3.9%) cases of metastatic disease and 0 deaths. Kaplan Meier analysis of metastasis free survival and overall survival yielded no statistically significant differences between the two treatment groups. Analysis of a large number of features of the two groups suggested no difference except more rubeotic eyes in the enucleated group (24%) than in the OAC group (6%).

Conclusions: In this single institution retrospective study of advanced intraocular retinoblastoma there was significantly more orbital retinoblastoma in the primarily enucleated group. OAC for advanced intraocular retinoblastoma does not increase the chance of orbital recurrence or metastatic disease compared to primary enucleation.