Retinal vasoproliferative tumor with total retinal detachment managed with plaque radiotherapy

Introduction

A 22-year-old female developed painless vision loss right eye (OD). Visual acuity was counting fingers OD, 20/20 OS. The OS was entirely normal. Examination of OD disclosed an orange-colored retinal mass located inferotemporally, measuring 7 mm in basal diameter and 4 mm in thickness [Figure 1]. There was a shallow nonrhegmatogenous total retinal detachment, intraretinal/subretinal exudation, preretinal hemorrhage and macular edema. The features were consistent with a retinal vasoproliferative tumor (VPT) and treatment with Iodine I-125 plaque radiotherapy with a dose of 4000 cGy was provided. Eleven months later, visual acuity had improved to 20/30 OD with tumor involution and resolution of subretinal fluid and macular edema. Four years later, visual acuity was 20/20 with stable findings and complete regression of the VPT to a 2.0-mm thick fibrous nonvascular mass [Figure 2]. There was no evidence of radiation maculopathy or papillopathy.

Figure 1.

Shallow exudative retinal detachment from inferotemporal active vasoproliferative tumor (a), with macular detachment and mild edema is noted on optical coherence tomography (b), and an echogenic mass of 4.0-mm thickness by ultrasonography(c)

Figure 2.

At 11 months following plaque radiotherapy, the detachment resolved and visual acuity returned to 20/30. At 4-year follow-up the retina remained flat with fibrosis of the tumor, and minimal retinal pigment epithelial alterations (a). The macula was slightly thinned on optical coherence tomography (b) but with 20/20visual acuity, and ultrasonography (c) disclosed reduced thickness to 2.0 mm

Comment

Retinal VPT has become a widely recognized entity in recent years.[1–5] In 1983, 12 cases were reported under the name presumed acquired retinal hemangioma, and the clinical features were thoroughly described.[1] In 1995, the same group reported 129 VPTs in 103 patients and classified this lesion into primary and secondary types.[2] This benign vascular mass has no predilection for gender, generally found in the inferotemporal periphery, and it can lead to serious complications including vision loss from subretinal fluid, exudation and retinal edema.[1–3] Management includes observation for asymptomatic lesions or laser photocoagulation, cryotherapy, photodynamic therapy or plaque radiotherapy for symptomatic tumors.[1–5]

Cohen and associates reviewed 30 eyes with a VPT treated with Iodine I-125 plaque radiotherapy and found tumor regression in 97% of cases, resolution of retinal detachment in 65% and improvement of visual acuity to 20/40 or better in 37%.[4] In the case reported here, plaque radiotherapy was successful in tumor control and vision return.