Dewan Aditi, Saran Ravindra Kumar, Gupta Smriti Nagpal, Arya Deepanjali, Goel Ruchi
1 Govind Ballabh Pant Institute of Post Graduate Medical Education and Research, New Delhi, India.
2 Maulana Azad Medical College, New Delhi, India.
Int J Surg Pathol. 2017 Jun;25(4):368-373. doi: 10.1177/1066896917692098. Epub 2017 Feb 14.
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.
眼内神经胶质病变较为罕见，包括视网膜胶质增生、错构瘤和星形细胞瘤，室管膜瘤则更为少见。室管膜瘤是一种生长缓慢的神经胶质肿瘤，优先发生于中枢神经系统（CNS），偶尔也会出现在CNS以外的部位，迄今为止仅报道过2例原发性视网膜室管膜瘤。我们在此报告第三例此类病例，患者为一名20岁男性，表现为疼痛性失明眼。摘除的标本显示存在神经胶质肿瘤，细胞呈片状排列，还有少量真性菊形团和假菊形团，免疫组化特征与CNS常见部位的典型室管膜瘤相似。此外，存在充满血液的间隙和少量增生血管，这给诊断带来了挑战。所有视网膜神经胶质病变的GFAP和S100均呈阳性。因此，EMA免疫染色以及MIB-1标记指数对于鉴别室管膜瘤与其他眼内神经胶质病变可能至关重要。
