Cancer-associated retinopathy (CAR) is a rare paraneoplastic entity, and literature suggests rapidly progressive blindness despite treatment. We discuss a case where disease stabilization was obtained after initiation of an aggressive immunosuppressive regimen. 

43-year-old male with history of WHO grade 4 IDH mutant right frontal anaplastic glioma was treated with surgical resection s/p 6 cycles of radiation/temozolomide, followed by a dendritic vaccine trial with twelve monthly doses. Two years later, he developed vision changes, initially described as “floaters”,progressive restriction in visual fields, starting in the left eye and quickly progressing to the right eye. After extensive work up by multiple ophthalmologists, a paraneoplastic cancer-associated retinopathy was confirmed with positive retinal immunohistochemistry,antibodies for enolase and CA-2. At the time of treatment initiation, microperimetry testing confirmed he was completely blind in his left eye and had only approximately 15 degrees central vision in his right eye. Treatment began with intravitreal dexamethasone implants followed by 5 cycles of plasmapheresis (PLEX) followed by intravenous immunoglobulin (IVIg) at 0.4gm/kg x3 days and was continued on a dexamethasone taper. His vision continued to deteriorate on repeat 3-month-interval microperimetry. Due to the patient’s high functional baseline, we initiated an aggressive regimen of IVIg 1g/kg and rituximab 375mg/m2 every 2 weeks, cyclophosphamide 750mg/m2 monthly, and maintenance oral steroids with goal to maintain residual vision. Repeat microperimetry testing at 3-month and 6-month intervals after initiation on this regimen demonstrated stabilization of disease course.

CAR leads to progressive,rapid visual loss caused by rod/cone dysfunction with retinal degeneration and despite immunosuppressive therapies, the prognosis remains poor. This case highlights that aggressive synchronous use of multiple immunosuppressive agents may help achieve disease stabilization.

There are no standardized treatment guidelines yet, but this case suggests that with implementation of aggressive immunosuppressive regimen, we may significantly impede the pace of irreversible visual loss.