Retinal vasoproliferative tumors (VPTs) are rare, acquired vascular lesions that can threaten vision if left untreated. Understanding their clinical presentation, differential diagnoses, and management strategies is essential for ophthalmologists, particularly when distinguishing VPTs from other retinal vascular tumors such as hemangioblastomas. Advances in imaging and treatment modalities have improved detection and visual outcomes, although challenges remain in peripheral lesion visualization and management of secondary cases associated with inflammation or degenerative retinal disease.
Introduction
Retinal vascular tumors may be congenital—such as retinal cavernous hemangioma and retinal arteriovenous communications (Wyburn-Mason syndrome)—or acquired, including retinal hemangioblastoma and VPTs.1 The distinction between congenital and acquired forms relates to the integrity of retinal tight junctions: congenital tumors generally have intact tight junctions and therefore lack subretinal fluid or lipid exudates, whereas acquired tumors involve disruption of these junctions, leading to vascular leakage with associated subretinal fluid or lipid exudation. Retinal VPTs and retinal hemangioblastoma are both acquired tumors, each with characteristic clinical features and specific systemic associations.
Figure 1. Typical features of retinal vasoproliferative tumor. Fundus photograph (A) showing prominent retinal lipid exudation, mild vitreous hemorrhage, and ill-defined retinal mass (8.0 x 6.0 x 2.3 mm in the inferotemporal periphery). Fluorescein angiogram (B) reveals adjacent areas of retinal capillary non perfusion and telangiectasia. Mild associated epiretinal membrane was present (C).
VPT was first recognized as a distinct clinical entity in 1982 when Baines reported the combination of peripheral telangiectatic nodules and posterior fibrocellular membranes.2 Initially labeled as “presumed acquired retinal hemangiomas,”³ VPT has become the most widely used terminology.4 Histologically, the lesion consists of a mixture of glial cells, retinal pigment epithelial cells, macrophages, foreign body giant cells, and a network of capillaries with some larger blood vessels.5-7 Tumor formation is considered reactive rather than neoplastic and is described as a retinal reactive astrocytic tumor.8 The underlying cause is often unidentified (primary, 87%), with inflammation (uveitis) or degenerative disease (retinitis pigmentosa) observed in secondary cases (13%).4
Clinical Features
Primary VPTs are usually solitary, whereas secondary tumors tend to be multiple. Vasoproliferative tumors typically present in the third or fourth decade of life, with equal sex distribution.4 Secondary VPTs often occur at a younger age.9 Common presenting symptoms include reduced vision, photopsia, and metamorphopsia, although some cases are diagnosed incidentally. Clinically, VPT appears as a globular yellow-pink, vascular mass in the inferotemporal peripheral retina (Figure 1A). The lesion has direct communication with retinal vessels of normal caliber, in contrast to retinal hemangioblastomas, which exhibit dilated and tortuous feeder vessels. Salient differentiating features between VPT and hemangioblastoma are summarized in Table 1.10
Subretinal fluid, lipid exudation, hemorrhage, and gliosis may obscure the tumor. Associated macular fibrosis and edema can lead to visual loss. Peripheral location complicates imaging with fluorescein angiography, ultrasonography, and optical coherence tomography (OCT). On fluorescein angiography, VPT demonstrates early hyperfluorescence with increasing diffuse leakage in late phases (Figure 1B). Telangiectatic and microaneurysmal changes, as well as capillary dropout reminiscent of Coats disease, may be observed in surrounding areas.1
Treatment
Management depends on tumor size and activity.1 Small, asymptomatic, gliotic tumors may be observed periodically. Treatment is indicated if the lesion is “active,” defined by the presence of subretinal fluid, hemorrhage, or lipid exudation. Tumors ≤1.5 mm can be treated with laser photocoagulation applied directly to the tumor, surrounding retina, and feeder vessels. Slightly larger tumors (up to 4.5 mm) are best treated with transconjunctival cryotherapy using a triple freeze-thaw cycle. Large tumors with ill-defined margins due to extensive exudation are technically challenging to treat with cryotherapy; these are better managed with radiation therapy, including plaque brachytherapy (ruthenium or iodine)11,12 or CyberKnife stereotactic radiotherapy (Accuray).13,14 Sequelae such as radiation retinopathy manifesting as cystoid macular edema or retinal neovascularization should be anticipated. Posteriorly located tumors with well-defined margins are best managed with photodynamic therapy (Figure 1C).15-17
Intravitreal anti-VEGF therapy and dexamethasone implants (Ozurdex; AbbVie) may be used adjunctively with cryotherapy, photodynamic therapy, or radiation therapy to prevent macular edema.18,19 In secondary VPT associated with uveitis, concomitant control of inflammation is necessary.20
Prognosis
Even small VPTs can cause significant vision loss, emphasizing the need for timely management.1 Advanced cases may progress to neovascular glaucoma, occasionally necessitating enucleation. Secondary VPTs tend to have worse visual outcomes.9 RP
References
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20. Pichi F, Neri P, Agarwal A, et al. Vasoproliferative tumors in intermediate uveitis. Retina. 2020;40(9):1765-1773. doi:10.1097/IAE.0000000000002656