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

• Published in: International journal of surgical pathology 2017-06, Vol.25 (4), p.368-373
• Main Authors: Dewan, Aditi, Saran, Ravindra Kumar, Gupta, Smriti Nagpal, Arya, Deepanjali, Goel, Ruchi
• Format: Article
• Language: English
• Subjects: Biomarkers, Tumor - analysis; Ependymoma - diagnosis; Ependymoma - pathology; Humans; Male; Retinal Neoplasms - diagnosis; Retinal Neoplasms - pathology; Young Adult
• ISSN: 1066-8969; 1940-2465
• DOI: 10.1177/1066896917692098

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.