Massive Retinal Gliosis in Microphthalmic Globe With Cyst Masquerading as a Neoplasm

Ruchi Goel,Ayushi Agarwal,Shweta Raghav,Ravindra Saran,Akash Raut

doi:10.7759/cureus.15419

Abstract

We report a rare case of a bilaterally blind, 33-year-old male, who presented with rapidly progressive, painless swelling in the right orbit for two months, in the absence of ocular trauma. Suspecting an intraocular neoplasm, a contrast-enhanced MRI (CE-MRI) orbit was performed, which was suggestive of a right-sided superomedial eccentric soft-tissue lesion with bilateral rudimentary globes associated with cyst, hypoplastic optic nerves, and focal areas of calcification. Diffusion-weighted MRI demonstrated diffusion restriction and yielded an indeterminate value of apparent diffusion coefficient (ADC). A right enucleation with excision of the cyst was performed. Histopathological examination confirmed the diagnosis of a right-sided massive retinal gliosis (MRG) with bilateral microphthalmia with cyst. This case demonstrates the role of a detailed histopathological analysis along with immunohistochemistry (IHC) in differentiating MRG from a neoplasm.

Highlights

Microphthalmia with cyst is a relatively common congenital entity, which results from developmental arrest during the sixth to seventh week of gestation at the 7-14-mm stage of embryological growth [1]
Suspecting an intraocular neoplasm, a contrast-enhanced MRI (CE-MRI) orbit was performed, which was suggestive of a right-sided superomedial eccentric soft-tissue lesion with bilateral rudimentary globes associated with cyst, hypoplastic optic nerves, and focal areas of calcification
Histopathological examination confirmed the diagnosis of a rightsided massive retinal gliosis (MRG) with bilateral microphthalmia with cyst

Summary

Introduction

Microphthalmia with cyst is a relatively common congenital entity, which results from developmental arrest during the sixth to seventh week of gestation at the 7-14-mm stage of embryological growth [1]. Massive retinal gliosis (MRG), originally described by Von Hippel in 1905, represents a pseudoneoplastic, polyclonal proliferation of retinal glial cells [2] It is more common in children as compared to adults and is rarely seen in association with microphthalmia with cyst [3]. We report a case of an adult male with bilateral microphthalmia with cyst presenting with a sudden, rapidly growing mass on the right side, which was later diagnosed as MRG on the basis of histopathological and immunohistochemistry (IHC) studies. Histopathological examination of the solid area revealed spindle-shaped glial cells arranged in interlacing short fascicles with abundant eosinophilic fibrillary cytoplasm and numerous dilated blood vessels. Histopathological examination of a) solid area (2X black arrow scanner view) revealing b) spindle-shaped cells with eosinophilic fibrillary cytoplasm and prominent dilated blood vessels (20X), c) better delineated at higher power (40X). The patient was bilaterally blind and opted for a spectacle prosthesis

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Disclosures