Long Term Outcome and Histologic Findings of a Retinal Astrocytic Hamartoma Treated with Intravitreal Injection of Anti-VEGF: A Case Report.

Kinza T Ahmad,Benjamin T Rollins,Paul H Phillips,Hana A Mansour,Sami H Uwaydat,Sergio Pina Oviedo,Alexander A Bialasiewicz

doi:10.1155/2021/7500791

Abstract

Background To our knowledge, this is the first report to describe the histologic changes of a retinal astrocytic hamartoma (RAH) in a patient with tuberous sclerosis complex (TSC) treated with antivascular endothelial growth factor (anti-VEGF), as well as the longest anti-VEGF treatment that such a patient has received (3 years). Case Presentation. We present a case of a 20-year-old female with TSC who developed progressive growth of a papillary astrocytic hamartoma that caused significant retinal edema, vitreous hemorrhage, and neovascular glaucoma. The patient was initially treated with 25 intravitreal anti-VEGF injections about every 1-3 months, but eventually developed a blind painful eye from neovascular glaucoma. Histopathologic evaluation of the enucleated globe showed a peculiar difference of the tumor according to its topography, with features reminiscent of pilocytic astrocytoma at the optic nerve head and features reminiscent of subependymal giant cell astrocytoma at the retrolaminar optic nerve. We hypothesize that these changes occurred as a secondary effect of the anti-VEGF treatment. Conclusions Anti-VEGF agents may decrease the ophthalmologic complications of RAH. We recommend that this treatment should be started early and continued for a protracted time at regular and frequent intervals. Moreover, a combination of therapies might prove to be superior to monotherapy and should therefore be considered in aggressive retinal astrocytic hamartomas.

Highlights

Retinal astrocytic hamartomas (RAH) are benign stationary tumors commonly associated with tuberous sclerosis complex (TSC)
Examination of the enucleated specimen revealed the presence of a very vascular hamartoma of the optic nerve and surrounding retina with superficial vessels corresponding to the filiform vessels
The retrolaminar optic nerve showed a proliferation of large polygonal cells with abundant eosinophilic cytoplasm with variable vacuolization, oval to round nucleus, and prominent nucleolus that diffusely infiltrated through the nerve fibers (Figure 7(c), left and middle panels)

Summary

Introduction

Retinal astrocytic hamartomas (RAH) are benign stationary tumors commonly associated with tuberous sclerosis complex (TSC). We highlight the patient’s clinical course and the histopathologic findings of the enucleated eye and suggest alternatives in the management of similar future patients based on our experience with this patient, as well as a comprehensive review of the literature To our knowledge, this is the first report that describes the histologic changes of a. We present a case of a 20-year-old female with TSC who developed progressive growth of a papillary astrocytic hamartoma that caused significant retinal edema, vitreous hemorrhage, and neovascular glaucoma. Histopathologic evaluation of the enucleated globe showed a peculiar difference of the tumor according to its topography, with features reminiscent of pilocytic astrocytoma at the optic nerve head and features reminiscent of subependymal giant cell astrocytoma at the retrolaminar optic nerve We hypothesize that these changes occurred as a secondary effect of the anti-VEGF treatment. A combination of therapies might prove to be superior to monotherapy and should be considered in aggressive retinal astrocytic hamartomas

Results

Discussion

Conclusion