Wabbels Bettina, Demmler Anke, Seitz Johannes, Woenckhaus Matthias, Bloss Heinz-Georg, Lorenz Birgit
Department of Paediatric Ophthalmology, Strabismology and Ophthalmogenetics, University of Regensburg, Franz Josef Strauss Allee 11, 93053 Regensburg, Germany.
Graefes Arch Clin Exp Ophthalmol. 2004 Sep;242(9):741-8. doi: 10.1007/s00417-004-0905-z.
Adult malignant optic nerve gliomas are rare and rapidly fatal visual pathway tumours. They represent a clinical entity different from the more common childhood benign optic nerve gliomas, which are frequently associated with neurofibromatosis I.
A 61-year-old woman presented with rapidly progressing right vision loss, lower altitudinal visual field defect and papilloedema. MRI showed intraorbital and intracranial swelling of the right optic nerve. Resection of the intracranial part of the right optic nerve up to the chiasm revealed anaplastic astrocytoma grade III. Within 1 year, the patient died of leptomeningeal metastasis despite radiotherapy. Clinical and MRI evaluation of the left eye and optic nerve were normal at all times.
Unilateral adult malignant glioma of the optic nerve is exceptional. The final diagnosis was only confirmed by optic nerve biopsy. In the literature, only one patient has been reported with a unilateral tumour manifestation; he was lost to follow-up 3 months later. All other cases were bilateral. To date, 44 case reports of adult malignant optic nerve glioma have been published, either malignant astrocytoma or glioblastoma. These tumours can mimic optic neuritis in their initial presentation. The diagnosis is seldom made before craniotomy. On MRI images, malignant glioma cannot be distinguished from optic nerve enlargement due to other causes. Although radiotherapy appears to prolong life expectancy, all presently available treatment options (radiation, surgery, radio-chemotherapy) are of limited value. Most patients go blind and die within 1 or 2 years.
成人恶性视神经胶质瘤是罕见且迅速致命的视觉通路肿瘤。它们代表了一种与更常见的儿童良性视神经胶质瘤不同的临床实体，后者常与神经纤维瘤病I相关。
一名61岁女性出现右眼视力迅速下降、下方视野缺损和视乳头水肿。磁共振成像（MRI）显示右侧视神经眶内和颅内肿胀。切除右侧视神经直至视交叉的颅内部分显示为间变性星形细胞瘤三级。尽管进行了放疗，患者在1年内死于软脑膜转移。左眼和视神经的临床及MRI评估始终正常。
单侧成人恶性视神经胶质瘤很罕见。最终诊断仅通过视神经活检得以证实。文献中仅报道过1例单侧肿瘤表现的患者，3个月后失访。所有其他病例均为双侧。迄今为止，已发表了44例成人恶性视神经胶质瘤的病例报告，均为恶性星形细胞瘤或胶质母细胞瘤。这些肿瘤在初始表现时可类似视神经炎。开颅手术前很少能做出诊断。在MRI图像上，恶性胶质瘤无法与其他原因导致的视神经增粗相区分。尽管放疗似乎可延长预期寿命，但目前所有可用的治疗选择（放疗、手术、放化疗）价值有限。大多数患者在1或2年内失明并死亡。
