Intraocular Ependymoma With Blood-Filled Spaces: Neoplasm or a Reactive Process With Ependymal Differentiation—A Dilemma

Abstract

Intraocular glial lesions are rare and include retinal gliosis, hamartomas, and astrocytomas and rarely ependymomas. Ependymomas are slow-growing glial tumors preferentially arising in the central nervous system (CNS), occasionally presenting at sites outside the CNS, with only 2 cases of primary retinal ependymoma reported till date. We report herein the third such case of a 20-year-old male who presented with a painful blind eye. The enucleated specimen showed presence of a glial tumor with cells arranged in sheets as well as few true rosettes and pseudo-rosettes and an immunohistochemical profile similar to a classical ependymoma at usual sites in the CNS. Additionally, the presence of blood-filled spaces and few proliferating blood vessels made it a diagnostic challenge. All retinal glial lesions are positive for GFAP and S100. Therefore, immunostaining for EMA as well as the MIB-1-labeling index maybe vital in differentiating ependymomas from other intraocular glial lesions.

Optic Pathway–Hypothalamic Glioma Apoplexy: A Report of Two Cases and Systematic Review of the Literature

“…Most cases have been managed by evacuating the resultant hematoma with decompression of the tumor. However, some reports have carried enucleation of the affected optic nerve, as reported by Yanoff et al and Dawan et al ( 17 , 48 ).…”

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

“…Ependymoma is a glial tumor of the central nervous system. Histologically, the presence of pseudorosettes, ependymal differentiation, and luminal dotlike positivity for EMA on IHC along with a low MIB-1 index facilitates the diagnosis of an ependymoma [9]. In rare cases, an orbital teratoma presents as sudden-onset proptosis and appears variegated on palpation.…”