Unique and progressive retinal degeneration in a patient with cancer associated retinopathy

PURPOSE: To report clinical course of a patient with cancer-associated retinopathy (CAR) medicated by steroid therapy, focusing on retinal degeneration progression. OBSERVATIONS: A 67 years-old female patient, who had a surgical history of endometrial carcinoma with adjuvant chemotherapy, was referred to our hospitals for the complaints of sudden reduced visual acuity and visual field constriction in the right eye. Best corrected visual acuity (BCVA) was 0.4 and 1.0 in right and left eyes, respectively. Funduscopy showed almost normal appearance in both eyes. Fluorescein angiography showed slight fluorescein leakage from the optic disc in both eyes and an inferior arcade vessel in the right eye. Optical coherence tomography (OCT) images showed loss of ellipsoid zone (EZ) and thinning of outer retinal layers at the nasal area of the fovea in both eyes. Goldmann perimetry (GP) demonstrated several paracentral absolute scotomas with peripheral visual field constriction in the right eye, and a paracentral relative scotoma with preserved peripheral visual field in the left eye. Ten months after the first visit, retinopathy progressed in both eyes. Funduscopy indicated mild retinal degeneration along with arcade veins with white sheathing of retinal arteries. Slightly visible EZ at the fovea and loss of EZ and interdigitation zone and thinning of outer retinal layers at other areas were observed in OCT images from both eyes. GP showed no response in both eyes. Oral prednisolone therapy was started and gradually tapered over a 3-month period. Twelve and fifteen months after the first visit, BCVA, EZ at the fovea in OCT images, and visual field gradually improved, whereas retinal degeneration along arcade veins became apparent. CONCLUSIONS AND IMPORTANCE: We reported a patient with CAR who exhibited progressive retinal degeneration and good response to oral prednisolone therapy. This case expands the clinical spectrum of CAR.