Inayama Yoshiaki, Hanashi Miho, Yazawa Takuya, Mitsui Hideaki, Kimura Ayako, Ito Daizo
Department of Pathology, Yokohama City University, Yokohama, Japan.
Hum Pathol. 2005 Jun;36(6):702-5. doi: 10.1016/j.humpath.2005.04.013.
We report a rare case of massive retinal gliosis that developed in a 32-year-old woman who had been born with bilateral microphthalmia. The patient had recently noticed left ophthalmos and underwent total resection of the affected eyeball. Histologically, the vitreous body had been totally replaced by massively proliferated spindle cells, which had delicate fibrillary cytoplasm without nuclear atypia. Because the attenuated retinal pigment epithelium and intact sclera were preserved at the periphery of the tumor, the tumor was thought to be retinal in origin. Immunohistochemically, the spindle cells were strongly positive for glial fibrillary acidic protein and neuron-specific enolase and partly positive for S-100 protein. These findings led to a diagnosis of massive gliosis of the retina. Clonality analysis of the tumor using a human androgen receptor assay revealed the polyclonal nature of the proliferating spindle cells. This is the first documentation of the polyclonality of this disease.
我们报告了一例罕见的大量视网膜胶质增生病例，该病例发生在一名32岁先天性双侧小眼球的女性身上。患者最近发现左眼突出，并接受了患眼眼球全切术。组织学检查显示，玻璃体已完全被大量增生的梭形细胞取代，这些细胞具有纤细的纤维状细胞质，无核异型性。由于肿瘤周边保留了变薄的视网膜色素上皮和完整的巩膜，因此认为肿瘤起源于视网膜。免疫组织化学检查显示，梭形细胞胶质纤维酸性蛋白和神经元特异性烯醇化酶呈强阳性，S-100蛋白呈部分阳性。这些发现导致诊断为视网膜大量胶质增生。使用人类雄激素受体检测对肿瘤进行克隆性分析，结果显示增殖的梭形细胞具有多克隆性质。这是该疾病多克隆性的首次记录。
