Simultaneous Bilateral Ophthalmic Artery Chemosurgery for Bilateral Retinoblastoma (Tandem Therapy)

Abstract

Purpose : Ophthalmic Artery Chemosurgery (OAC) is effective for the management of naive or failed unilateral retinoblastoma (RB). Simultaneous initial management of bilateral disease with OAC (tandem therapy) was first reported 5 years ago but since then there have only been scattered case reports totaling fewer than 10 patients worldwide. The purpose of this study was to look at patient and ocular outcomes for naive bilateral patients from our institution who received tandem therapy.

Methods : Retrospective, IRB approved chart review of all bilateral RB patients naïve to treatment with OAC in both eyes initially managed with tandem therapy at Memorial Sloan Kettering Cancer Center (MSKCC) between May 2006 and May 2015. Severity of initial disease was assessed using International Classification of Retinoblastoma-Children’s Oncology Group (ICRb) and Reese Ellsworth (RE) eye groupings. Ocular survival, patient survival, systemic metastases, second tumors, ERG before and after treatment, and systemic adverse events (via the Common Terminology Criteria for Adverse Events version 4.03) were recorded and analyzed.

Results : A total of 122 eyes from 61 cases were included. Patient demographics, ocular groupings and treatment parameters are reported in Figure 1. Four eyes (4/122) came to enucleation. (3 for progressive disease, 1 family choice). ERG analysis before and after treatment showed 53% of ERG values were unchanged (increment or decrement of less than 25 µV), while 21.3% improved, and 25.4% decreased. There were no procedural deaths or strokes (1 TIA). Hospitalization and treatment for neutropenia with fever occurred at an average rate of 0.95% (2 cases). Transfusion of blood products secondary to anemia or thrombocytopenia occurred at an average rate of 1.9% (3 cases, 4 transfusions). There were no cases of metastatic RB. Second non-ocular cancer was present in 2 cases, both were pineoblastoma (1 deceased).

Conclusions : Tandem therapy is an effective technique for the initial management of bilateral retinoblastoma in patients naïve to treatment. Ocular survival was excellent, no patient died of metastatic disease and second cancers were not more common in this group. Despite bilateral treatment the rate of clinically significant bone marrow suppression and/or fever/neutropenia was under 2%.

This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.

 

Figure 1: Patient and Treatment Details