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

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.

A clinical and morphological analysis of a retinal and optic nerve astrocytic hamartoma case

A clinical and morphological description of a case of pilocytic astrocytoma (astrocytic hamartoma) of the retina and optic nerve in an 11-year old girl is presented. The clinical and instrumental characteristics of the tumor are determined by ultrasound and MRI tests. Detailed cytological and pathomorphological description of the tumor, which is rarely observed, is given. The tumor poses a threat to the eye and requires that the eyeball be removed.

Presumed retinal pericapillary astrocytic hamartoma: multimodal imaging findings of a novel hamartomatous lesion

PurposeTo describe the multimodal imaging findings of retinal lesions that clinically resemble retinal astrocytic hamartomas (RAHs), but also have unique characteristics that we believe represent a novel variant.MethodsObservational study. Five eyes in five patients with solitary retinal lesion evaluated at the retina division of three institutions. We describe the multimodal imaging findings including fundus photography, fundus autofluorescence, fluorescein angiography, spectral-domain optical coherence tomography (OCT), swept-source OCT, swept-source OCT angiography and ultrasonography.ResultsThe retinal lesions described shared similar appearance to RAHs but demonstrated unique features such as glistening granular appearance on fundus photographs with perivascular hyperreflectivity with OCT and OCT angiography.ConclusionThe lesions described herein appear to have unique characteristics that warrant a designation as a novel RAH variant. The name presumed retinal pericapillary astrocytic hamartoma is suggested.