Woog J J, Albert D M, Craft J, Silberman N, Horns D
Graefes Arch Clin Exp Ophthalmol. 1983;220(1):25-31. doi: 10.1007/BF02307012.
A twenty-one year old female with previously unsuspected neurofibromatosis presented for evaluation of a blind painful eye. Histopathologic examination of the enucleated specimen revealed choroidal thickening with ovoid bodies and proliferation of connective tissue with pigment-containing cells and ganglion-like cells. Electron microscopic study of the latter cell population revealed typical morphologic features of ganglion cells, including numerous electron-dense intracellular granules and an abundance of mitochondria and rough endoplasmic reticulum. These pathologic findings were interpreted as consistent with the diagnosis of choroidal ganglioneuroma occurring in the context of ocular neurofibromatosis. The literature concerning this unusual tumor is reviewed and the possible relationship of this lesion to neurofibromatosis and other disorders of neural cresent proliferation are briefly discussed.
一名21岁女性，此前未被怀疑患有神经纤维瘤病，因一只失明且疼痛的眼睛前来评估。对摘除眼球标本的组织病理学检查显示脉络膜增厚，伴有卵形小体，结缔组织增生，含有色素细胞和神经节样细胞。对后一种细胞群的电子显微镜研究揭示了神经节细胞的典型形态特征，包括大量电子致密的细胞内颗粒以及丰富的线粒体和粗面内质网。这些病理结果被解释为与眼神经纤维瘤病背景下发生的脉络膜神经节神经瘤的诊断一致。本文回顾了有关这种不寻常肿瘤的文献，并简要讨论了该病变与神经纤维瘤病及其他神经嵴增殖性疾病的可能关系。
