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Clinical Sciences |

Retinal Vasoproliferative Tumors:  Comparative Clinical Features of Primary vs Secondary Tumors in 334 Cases

Carol L. Shields, MD; Swathi Kaliki, MD; Saad Al-Dahmash, MD; Duangnate Rojanaporn, MD; Shripaad Y. Shukla, MD; Brad Reilly, BS; Jerry A. Shields, MD
JAMA Ophthalmol. 2013;131(3):328-334. doi:10.1001/2013.jamaophthalmol.524.
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Objective  To compare the clinical features of primary vs secondary retinal vasoproliferative tumors (VPTs).

Methods  Retrospective case series of 334 tumors in 295 eyes of 275 patients.

Results  Of 275 patients with VPT, 41% (n = 113) were male and 59% (n = 162) were female, with a mean age of 44 years at presentation. Primary VPT occurred in 80% (n = 219) and secondary VPT, in 20% (n = 56) of patients. Secondary VPT (n = 67) occurred in eyes with retinitis pigmentosa (n = 15, 22%), pars planitis (n = 14, 21%), Coats disease (n = 11, 16%), previous retinal detachment surgery (n = 8, 12%), idiopathic peripheral retinal vasculitis (n = 4, 6%), familial exudative vitreoretinopathy (n = 3, 4%), and others (n = 12, 18%). The mean interval between diagnosis of underlying ocular condition and secondary VPT was 160 months. Statistically significant differences (P < .05) in clinical features (primary vs secondary VPTs) included mean age at presentation (46 vs 38 years), visual symptoms (74% vs 87%), poor visual acuity worse than 20/200 (15% vs 28%), bilaterality (4% vs 20%), multifocality (5% vs 15%), postequatorial tumor location (20% vs 33%), tumor basal dimension (6 vs 7 mm), anterior chamber cells (16% vs 30%), and vitreous cells (19% vs 48%).

Conclusions  Retinal vasoproliferative tumor can be primary (80%) or secondary (20%). Compared with primary VPT, secondary VPT is more often bilateral, multiple, and larger and occurs at an earlier age associated with poorer visual acuity.

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Figure 1. Primary retinal vasoproliferative tumor showing various features of intraretinal exudation (A-D and E), subretinal/intraretinal hemorrhage (A, B, D, E, and F), shallow subretinal fluid (A, B, D, and E), and retinal pigment epithelial alterations (A and C).

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Figure 2. Secondary retinal vasoproliferative tumor with underlying conditions of retinitis pigmentosa (A), postretinal detachment surgery (B), retinopathy of prematurity (C), and pars planitis (D).

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Figure 3. Primary retinal vasoproliferative tumor before (A) and after (B) cryotherapy.

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