Stern J, Jakobiec F A, Housepian E M
Arch Ophthalmol. 1980 Mar;98(3):505-11. doi: 10.1001/archopht.1980.01020030501014.
A retrospective clinicopathologic study of 34 patients with optic nerve glioma disclosed two important architectural tumor forms: circumferential-perineural pattern featuring tumor eruption and proliferation in the subarachnoid space correlating with the presence of neurofibromatosis, and an expansile-intraneural pattern correlating with the absence of neurofibromatosis. The 18 patients with neurofibromatosis had a mean age of 4.9 years at presentation and tended to be younger than the 16 patients without neurfibromatosis (mean age, 12 years). Instead of "arachnoidal hyperplasia," electron microscopic studies in the electron microscopic studies in the circumferential-perineural pattern showed tumor astrocytes admixed with reactive meningothelial cells, fibroblasts, and collagen in distended subarachnoid space. Florid invasion of the leptomeninges (arachnoidal gliomatosis) exhibited by many optic nerve gliomas in neurofibromatosis suggests that they are true neoplasms rather than hamartomas, but their frequent location in the distal anterior visual pathway often confers a good clinical prognosis.
一项对34例视神经胶质瘤患者的回顾性临床病理研究揭示了两种重要的肿瘤结构形式：圆周-神经周模式，其特征为肿瘤在蛛网膜下腔喷发和增殖，与神经纤维瘤病的存在相关；以及膨胀-神经内模式，与神经纤维瘤病的不存在相关。18例患有神经纤维瘤病的患者就诊时的平均年龄为4.9岁，往往比16例没有神经纤维瘤病的患者（平均年龄12岁）更年轻。在圆周-神经周模式的电镜研究中，并非“蛛网膜增生”，而是显示在扩张的蛛网膜下腔中肿瘤星形胶质细胞与反应性脑膜内皮细胞、成纤维细胞和胶原混合。神经纤维瘤病中许多视神经胶质瘤表现出的柔脑膜的显著浸润（蛛网膜胶质瘤病）表明它们是真正的肿瘤而非错构瘤，但它们在视路前段远端的常见位置通常赋予良好的临床预后。
