Article: Peripapillary subretinal neovascularization in retinoblastoma

Background: Peripapillary subretinal neovascularization (PSRNV) is a rare type of choroidal neovascularization. Herein we report a case of retinoblastoma complicating PSRNV, and discuss the histopathological findings.

Methods: A 1-year-old male underwent enucleation of his right eyeball based on the clinical diagnosis of bilateral retinoblastoma after chemotherapy.

Results: There was a mass arising from the retina showing highly calcified and necrotic retinoblastoma. The peripapillary region revealed neovascular membrane extending from the optic nerve head to the subretinal space. The membrane included retinal pigment epithelial (RPE) cells and glial cells, as well as endothelial cells. Immunohistochemistry revealed cytokeratin 18-positive RPE cells situated beneath glial fibrillary acidic protein-positive glial cells and their processes. The neovascular membrane did not have a connection with vessels arising from the optic nerve head. There were multiple mound foci made up of proliferated RPE cells in the globe.

Conclusion: These results suggest that migration of RPE cells and glial cells plays a crucial role in the pathogenesis of PSRNV, which might be directly or indirectly mediated by retinoblastoma.

Citing Articles

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Jo D, Kim J, Kim J Adv Exp Med Biol. 2021; 1296:349-358.

PMID: 34185303 DOI: 10.1007/978-3-030-59038-3_21.

References

1.

Viet Tran H, Bovey E, Uffer S, Zografos L . Peripapillary choroidal neovascularization associated with melanocytoma of the optic disc: a clinicopathologic case report. Graefes Arch Clin Exp Ophthalmol. 2006; 244(10):1367-9. DOI: 10.1007/s00417-006-0270-1. View

2.

Hisatomi T, Enaida H, Sakamoto T, Kanemaru T, Kagimoto T, Yamanaka I . Cellular migration associated with macular hole: a new method for comprehensive bird's-eye analysis of the internal limiting membrane. Arch Ophthalmol. 2006; 124(7):1005-11. DOI: 10.1001/archopht.124.7.1005. View

3.

Sullu Y, Yildiz L, Erkan D . Submacular surgery for choroidal neovascularization secondary to optic nerve drusen. Am J Ophthalmol. 2003; 136(2):367-70. DOI: 10.1016/s0002-9394(03)00187-9. View

4.

Peer J, Neufeld M, Baras M, Gnessin H, Itin A, Keshet E . Rubeosis iridis in retinoblastoma. Histologic findings and the possible role of vascular endothelial growth factor in its induction. Ophthalmology. 1997; 104(8):1251-8. DOI: 10.1016/s0161-6420(97)30150-x. View

5.

Lopez P, Green W . Peripapillary subretinal neovascularization. A review. Retina. 1992; 12(2):147-71. View

6.

Jin M, Chen Y, He S, Ryan S, Hinton D . Hepatocyte growth factor and its role in the pathogenesis of retinal detachment. Invest Ophthalmol Vis Sci. 2003; 45(1):323-9. DOI: 10.1167/iovs.03-0355. View

7.

Browning D, Fraser C . Ocular conditions associated with peripapillary subretinal neovascularization, their relative frequencies, and associated outcomes. Ophthalmology. 2005; 112(6):1054-61. DOI: 10.1016/j.ophtha.2004.11.062. View

8.

Kase S, Yoshida K, Ohgami K, Shiratori K, Harada T, Ohno S . Expression of p27(KIP1) and cell proliferation in human retina and retinoblastoma. Anticancer Res. 2005; 25(6B):3843-6. View

9.

Campochiaro P, Soloway P, Ryan S, Miller J . The pathogenesis of choroidal neovascularization in patients with age-related macular degeneration. Mol Vis. 1999; 5:34. View

10.

Shuler Jr R, Hubbard 3rd G, Grossniklaus H . Retinal neovascularization associated with retinoblastoma. Am J Ophthalmol. 2005; 139(1):210-2. DOI: 10.1016/j.ajo.2004.07.033. View

11.

Shibuya M, Okamoto H, Nozawa T, Utsumi J, Reddy V, Echizen H . Proteomic and transcriptomic analyses of retinal pigment epithelial cells exposed to REF-1/TFPI-2. Invest Ophthalmol Vis Sci. 2007; 48(2):516-21. DOI: 10.1167/iovs.06-0434. View

Peripapillary Subretinal Neovascularization in Retinoblastoma