Vasoproliferative Tumors of the Retina: Pathophysiology, Clinical Features, and Treatment Approaches.

BACKGROUND: Retinal vasoproliferative tumors (RVPTs) are rare, benign lesions appearing as elevated, pink masses in the peripheral retina. Initially considered acquired retinal capillary hemangioblastomas, RVPTs are now recognized as distinct entities, with idiopathic and secondary forms. Though primarily affecting individuals between 30 and 50 years of age, their pathogenesis remains under investigation.

SUMMARY: Recent histopathological evidence suggests RVPTs have a predominantly glial rather than vascular origin. Clinically, RVPTs cause visual deterioration, floaters, and photopsia, often with subretinal/intraretinal exudation, epiretinal membranes, vitreous hemorrhage, or retinal detachment. Fluorescein angiography reveals telangiectatic vessels with intense late-phase hyperfluorescence. Secondary RVPTs comprise up to 84% of cases, linked to conditions such as Coats' disease, uveitis, and toxoplasmosis. Management depends on tumor size, location, and complications. Small, asymptomatic lesions may be observed, while vision-threatening cases require intervention. Treatment options include cryotherapy, laser photocoagulation, photodynamic therapy, intravitreal anti-vascular endothelial growth factor/corticosteroid injections, plaque brachytherapy, and vitreoretinal surgery. While some tumors remain stable without treatment, surgical interventions, particularly pars plana vitrectomy, effectively control complications and tumor activity.

KEY MESSAGES: The evolving understanding of RVPT pathogenesis necessitates multicenter studies to establish standardized diagnostic and therapeutic guidelines. Integrating histopathological insights with clinical findings will optimize management strategies and improve patient outcomes for these rare retinal tumors.