Ahead of print

Original article

Clinical Features and Therapeutic Alternatives in Eyes with Secondary Vasoproliferative Tumors: A Single-Center Turkish Perspective

Full Text links

Summary

Aims: To evaluate retrospectively the clinical characteristics and therapeutic outcomes in eyes with secondary vasoproliferative retinal tumors (VPRT) at a tertiary center in Turkey.

Material and Methods: Patients diagnosed with secondary VPRT between 2005 and 2025 with a follow-up of 6 months or more were included. 24 eyes of 21 patients were evaluated. Their charts and multimodal imaging features were analyzed, and treatments noted.

Results: Mean age was 26.1 years (6–57). Mean follow-up was 61.1 months (6–151). Baseline BCVA was 0.77 LogMAR (0.00–2.30). VPRT was linked to non-infectious uveitis in 12 patients, retinitis pigmentosa in 5 patients, X-linked Juvenile Retinoschisis, Coats’ disease, retinal dystrophy and retinopathy of prematurity in 1 patient each. The inferotemporal quadrant was the most common site (66.7%). Treatments included dexamethasone implant, transscleral cryotherapy, systemic immunosuppressive therapy, laser photocoagulation, CyberKnife, intravitreal anti-VEGF, vitreoretinal surgery, photodynamic therapy, intravitreal triamcinolone acetonide and enucleation. At baseline, 15 eyes had macular intraretinal fluid. At the final visit, dry macula was achieved in 9 of 15 eyes. Mean final BCVA was 1.01 LogMAR (0.00–3.00). Two eyes deteriorated to no light perception. One was enucleated.

Conclusion: This largest cohort of patients from Turkey with secondary VPRT reflects the diverse underlying etiologic associations and heterogeneous clinical manifestations at a tertiary center. Various therapeutic techniques were required to cope with this difficult-to-treat, challenging disease entity.

References

  1. Shields JA, Decker WL, Sanborn GE, Augsburger JJ, Goldberg RE. Presumed acquired retinal hemangiomas. Ophthalmology. 1983;90:1292-1300. doi: 10.1016/S0161-6420(83)34389-X
  2. Shields CL, Shields JA, Barrett J, De Potter P. Vasoproliferative tumors of the ocular fundus: classification and clinical manifestations in 103 patients. Arch Ophthalmol. 1995;113:615-623. doi: 10.1001/archopht.1995.01100050083035
  3. Shields CL, Kaliki S, Al-Dahmash S, et al. Retinal vasoproliferative tumors: comparative clinical features of primary vs secondary tumors in 334 cases. JAMA Ophthalmol. 2013;131:328-334. doi: 10.1001/2013.jamaophthalmol.524
  4. Kiri H, Raval V, Ali H, Das AV, Kaliki S. Vasoproliferative retinal tumors: clinical presentation and treatment outcome. Eur J Ophthalmol. 2023;33:1596-1603. doi: 10.1177/11206721221149075
  5. Walinjkar JA, Sharma U, Rishi P, Rishi E, Gopal L, Sharma T. Clinical features and treatment outcomes of vasoproliferative tumors in Indian participants. Indian J Ophthalmol. 2018;66:246-251. doi: 10.4103/ijo.IJO_210_17
  6. Garcia-Arumi J, Distefano LN, Fonollosa A, Quijano C, Corcostegui B. Management of vision-threatening complications of vasoproliferative tumors of the retina. Ophthalmic Res. 2015;54:34-40. doi: 10.1159/000430955
  7. Cebeci Z, Oray M, Tuncer S, Tugal Tutkun I, Kir N. Intravitreal dexamethasone implant (Ozurdex) and photodynamic therapy for vasoproliferative retinal tumours. Can J Ophthalmol. 2014;49:e83-84. doi: 10.1016/j.jcjo.2014.04.006
  8. Kumar K, Raj P, Chandnani N, Agarwal A. Intravitreal dexamethasone implant with retinal photocoagulation for adult-onset Coats’ disease. Int Ophthalmol. 2019;39:465-470. doi: 10.1007/s10792-018-0827-0
  9. Faria Pereira A, Teixeira-Martins R, Rocha-Sousa A, Penas S. Vasoproliferative retinal tumors: manifestations, management, and outcomes in a case series. Case Rep Ophthalmol. 2024;16:27-36. doi: 10.1159/000542956
  10. Elnahry AG, Bellur S, Wiley HE, Sen HN, Kodati S. Treatment of intermediate uveitis-associated retinal vasoproliferative tumors: a case series. Retin Cases Brief Rep. 2023. doi: 10.1097/ICB.0000000000001494
  11. Ie A, Sagoo MS, MacLaren RE, Cehajic-Kapetanovic J. Retinal focal nodular gliosis (vasoproliferative tumors) have varied clinical courses requiring tailored management: a case series. Ocul Oncol Pathol. 2024;10:175-181. doi: 10.1159/000538984
  12. Martínez-Castillo S, Gallego-Pinazo R, Dolz-Marco R, Marín-Lambíes C, Díaz-Llopis M. Adult Coats’ disease successfully managed with the dexamethasone intravitreal implant (Ozurdex®) combined with retinal photocoagulation. Case Rep Ophthalmol. 2012;3:123-127. doi: 10.1159/000337481
  13. Subramanian B, Nangia P, Rishi P, et al. Clinical profiles of retinal vasoproliferative tumors. J Vitreoretin Dis. 2025;9:198-203. doi: 10.1177/24741264241296464
  14. Manjandavida FP, Shields CL, Kaliki S, Shields JA. Cryotherapy-induced release of epiretinal membrane associated with retinal vasoproliferative tumor: analysis of 16 cases. Retina. 2014;34:1644- 1650. doi: 10.1097/IAE.0000000000000137
  15. Pichi F, Neri P, Agarwal A, et al. Vasoproliferative tumors in intermediate uveitis. Retina. 2020;40:1765-1773. doi: 10.1097/IAE.0000000000002656
  16. Ahmed AS, Dutta Majumder P, Elizabeth A, Rao V, Ganesh S, Biswas J. Retinal vasoproliferative tumours in uveitis. Ocul Immunol Inflamm. 2024;32:2056-2060. doi: 10.1080/09273948.20 24.2317418
  17. Schmelter V, Fuerweger C, Muacevic A, Priglinger SG, Foerster P, Liegl R. Robotic-assisted radiosurgery for the treatment of vasoproliferative tumours. Acta Ophthalmol (Copenh). 2021;99:456-460. doi: 10.1111/aos.14609
  18. Mares V, Veloso CE, Pulido JS, Nehemy MB. Surgical outcomes of vasoproliferative retinal tumors refractory to noninvasive therapies. Retina. 2022;42:1772-1779. doi: 10.1097/IAE.0000000000003520