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Clinical Sciences
March 2013

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

Author Affiliations

Author Affiliations: The Ocular Oncology Service (Drs C. L. Shields, Kaliki, Al-Dahmash, Rojanaporn, Shukla, and J. A. Shields and Mr Reilly), Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania; Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia (Dr Al-Dahmash); and Department of Ophthalmology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand (Dr Rojanaporn).

JAMA Ophthalmol. 2013;131(3):328-334. doi:10.1001/2013.jamaophthalmol.524

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.