Abstract

To report the clinicopathological findings of retinal vasoproliferative tumor/reactive retinal astrocytic tumor (VPT/RRAT) with retinal vasculitis, treated by tumor resection. A retrospective single case report. A 29-year-old Japanese woman was referred with cystoid macular edema and retinal vasculitis in the right eye. Best-corrected visual acuity (BCVA) was 0.9. Fundus examination, optical coherence tomography, and fluorescein angiography demonstrated VPT/RRATs in the temporal retina surrounded by a subretinal exudate, serous retinal detachment and macular edema, and retinal vasculitis. Despite 3 months of oral prednisolone treatment, a full-thickness macular hole developed. Pars plana vitrectomy and endoresection of the VPT/RRATs were performed. Pathological and immunohistochemical analyses with anti-CD34 antibody, anti-glial fibrillary acidic protein (GFAP) antibody, anti-Ki67 antibody, and anti-vascular endothelial growth factor (VEGF) antibody were conducted on the excised tissue. Inflammation was evaluated by immunohistological staining with leucocyte common antigen (LCA), anti-CD3 antibody, and anti-CD20 antibody. After surgery, the macular hole closed, BCVA improved to 1.2, retinal vasculitis was ameliorated, and retinal exudate disappeared. There was no recurrence of VPT/RRAT or retinal vasculitis. Pathological examination showed that GFAP and VEGF were widely expressed, irrespective of the distribution of blood vessels. Ki67-positive proliferating cells were detected in the perivascular area. LCA-positive leukocytes and CD3-positive T cells were detected throughout the samples, whereas CD20-positive B cells were rarely detected. Endoresection of VPT/RRAT could be a good treatment option for secondary VPT/RRAT accompanied by retinal vasculitis. Pathological findings revealed for the first time that inflammatory cells infiltrate the tissue in secondary VPT/RRAT.

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