Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, USA.
Am J Ophthalmol. 2013 Mar;155(3):593-608.e1. doi: 10.1016/j.ajo.2012.09.002. Epub 2012 Dec 6.
To evaluate the cellular nature of and diagnostic terminology used in connection with acquired retinal "vasoproliferative tumors."
Retrospective clinicopathologic study.
Clinical records and microscopic slides of 4 enucleated globes were reviewed. Special stains and immunohistochemical probes for CD31, CD34, p53, glial fibrillary acidic protein (GFAP), CD163, and Ki67 (cell replication) were employed; ultrastructural and fluorescence in situ hybridization (FISH) analyses were performed.
Tumors were located inferotemporally in middle-aged patients. They were uniformly composed of compacted elongated, GFAP-positive spindle cells (due to intermediate filaments identified ultrastructurally) with a Ki67 index of less than 1%. Rosenthal fibers and eosinophilic granular bodies were observed. Hyalinized periodic acid-Schiff-positive vessels were widely separated. CD31 and CD34 revealed a sparse microvasculature. Tumor-associated exudate spread predominantly subretinally. The retinal pigment epithelium had undergone extensive placoid fibrous metaplasia with focal ossification. P53 upregulation, BRAF-KIAA gene rearrangement, and IDH1R132H mutation typically associated with low-grade astrocytic neoplasms were absent.
Retinal "vasoproliferative" tumors have been mischaracterized, because they actually display a paucity of microvessels. Proliferating fibrous astrocytes with a very low proliferation index predominate, without immunohistochemical or genetic evidence favoring a neoplasm. Subretinal exudate appeared capable of provoking widespread fibrous metaplasia of the pigment epithelium that was mainly responsible for secondary retinal damage. The term "reactive retinal astrocytic tumor" is proposed as more appropriate for this entity. In carefully selected progressive lesions, consideration should be given to earlier surgical intervention before extensive subretinal exudate accumulates and pigment epithelial proliferation with fibrous metaplasia ensues.
评估获得性视网膜“血管增生性肿瘤”的细胞特征和诊断术语。
回顾性临床病理研究。
回顾了 4 例眼球摘除标本的临床记录和显微镜载玻片。使用了特殊染色和 CD31、CD34、p53、胶质纤维酸性蛋白（GFAP）、CD163 和 Ki67（细胞复制）的免疫组织化学探针；进行了超微结构和荧光原位杂交（FISH）分析。
肿瘤位于中年患者的颞下象限。它们均由 Compacted 拉长的、GFAP 阳性的梭形细胞组成（由于超微结构中识别的中间丝），Ki67 指数小于 1%。观察到 Rosenthal 纤维和嗜酸性颗粒体。玻璃样酸性 PAS 阳性血管广泛分离。CD31 和 CD34 显示稀疏的微血管。肿瘤相关渗出物主要在视网膜下扩散。视网膜色素上皮经历了广泛的斑块状纤维化生，伴有局灶性骨化。低级别星形细胞瘤通常与 p53 上调、BRAF-KIAA 基因重排和 IDH1R132H 突变无关。
视网膜“血管增生性”肿瘤被错误描述，因为它们实际上显示出很少的微血管。增生的纤维星形细胞占主导地位，增殖指数非常低，没有免疫组织化学或遗传证据支持肿瘤。视网膜色素上皮的广泛纤维化生可能导致继发性视网膜损伤，而视网膜下渗出物似乎能够引发这种变化。提出“反应性视网膜星形细胞瘤”作为更适合这种实体的术语。在精心选择的进行性病变中，应考虑在广泛的视网膜下渗出物积聚和伴有纤维化生的色素上皮增生之前进行早期手术干预。
