Pathology characteristics of ocular von Hippel-Lindau disease with neovascularization of the iris and cornea: a case report.
Pathology characteristics of ocular von Hippel-Lindau disease with neovascularization of the iris and cornea: a case report.
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DOI：
10.1186/s13256-015-0539-2
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发表时间：
2015-03-25
影响因子：
1
通讯作者：
Chan CC
中科院分区：
文献类型：
--
作者：
Chen S;Chew EY;Chan CC
视网膜血管母细胞瘤是von Hippel-Lindau病最常见的肿瘤之一。除了von Hippel-Lindau病的典型病理特征外，我们首次报告了一种严重而罕见的眼部并发症，其特征是角膜和虹膜中的新生血管。一位41岁白人男性，长期患有视网膜血管母细胞瘤，表现为虹膜和角膜新生血管，并伴有角膜穿孔。他的右眼失明和疼痛，导致决定摘除眼球。显微镜下，摘除眼球显示角膜和虹膜新生血管。角膜穿孔，伴有排出性出血并挤压眼内内容物，包括视网膜。视网膜大血管母细胞瘤位于视神经头附近的后极。肿瘤主要由大细胞组成，胞浆呈泡沫状。骨形成也是存在的。我们的病理结果与先前描述的视网膜血管母细胞瘤的特征一致。这个病例很少见，因为虹膜和角膜中同时存在新生血管，这可能导致角膜穿孔和视力丧失。
Retinal hemangioblastoma is one of the most common tumors in von Hippel-Lindau disease. In addition to the classical pathological characteristics of von Hippel-Lindau disease, we report, for what we believe to be the first time, a severe and rare ocular complication characterized by neovascularization in the cornea and iris. A 41-year-old white man with a long history of retinal hemangioblastoma presented with neovascularization of his iris and cornea as well as corneal perforation. His right eye was blind and painful, leading to a decision of enucleation. On microscopy, the enucleated eye showed neovascularization of the cornea and iris. The cornea was perforated with an expulsive hemorrhage and extruding intraocular contents, including the retina. A large retinal hemangioblastoma was located at the posterior pole adjacent to the optic nerve head. The tumor was mainly composed of large cells with foamy cytoplasm. Bone formation was also present. Our pathology findings were consistent with previously described features of retinal hemangioblastoma. The present case is unusual because of the co-existing neovascularization in the iris and cornea, which may have led to corneal perforation and vision loss.