Parsa C F, Hoyt C S, Lesser R L, Weinstein J M, Strother C M, Muci-Mendoza R, Ramella M, Manor R S, Fletcher W A, Repka M X, Garrity J A, Ebner R N, Monteiro M L, McFadzean R M, Rubtsova I V, Hoyt W F
Wilmer Ophthalmological Institute, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Arch Ophthalmol. 2001 Apr;119(4):516-29. doi: 10.1001/archopht.119.4.516.
To demonstrate spontaneous regression of large, clinically symptomatic optic pathway gliomas in patients with and without neurofibromatosis type 1 (NF-1).
Patient cases were collected through surveys at 2 consecutive annual meetings of the North American Neuro-Ophthalmology Society (NANOS) and through requests on the NANOSNET Internet listserv. Serial documentation of tumor signal and size, using magnetic resonance imaging in 11 patients and computed tomography in 2 patients, was used to evaluate clinically symptomatic optic pathway gliomas. All tumors met radiologic criteria for the diagnosis of glioma and 4 patients had biopsy confirmation of their tumors. In 3 patients, some attempt at therapy had been made many years before regression occurred. In one of these, radiation treatment had been given 19 years before tumor regression, while in another, chemotherapy had been administered 5 years before signal changes in the tumor. In the third patient, minimal surgical debulking was performed 1 year before the tumor began to shrink.
Spontaneous tumor shrinkage was noted in 12 patients. Eight patients did not have NF-1. In an additional patient without NF-1, a signal change within the tumor without associated shrinkage was detected. Tumor regression was associated with improvement in visual function in 10 of 13 patients, stability of function in 1, and deterioration in 2.
Large, clinically symptomatic optic gliomas may undergo spontaneous regression. Regression was seen in patients with and without NF-1. Regression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging. A variable degree of improvement in visual function may accompany regression. The possibility of spontaneous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors.
证明1型神经纤维瘤病（NF-1）患者和非NF-1患者中大型有临床症状的视路胶质瘤的自发消退。
通过在北美神经眼科学会（NANOS）连续两届年会上的调查以及通过NANOSNET互联网邮件列表上的请求收集患者病例。使用11例患者的磁共振成像和2例患者的计算机断层扫描对肿瘤信号和大小进行系列记录，以评估有临床症状的视路胶质瘤。所有肿瘤均符合胶质瘤的放射学诊断标准，4例患者经活检证实肿瘤。在3例患者中，在消退发生前很多年曾尝试过一些治疗。其中1例在肿瘤消退前19年接受了放射治疗，另一例在肿瘤信号改变前5年接受了化疗。第3例患者在肿瘤开始缩小前1年进行了最小限度的手术减瘤。
12例患者出现肿瘤自发缩小。8例患者没有NF-1。在另外1例没有NF-1的患者中，检测到肿瘤内有信号改变但无相关缩小。13例患者中有10例肿瘤消退与视觉功能改善相关，1例功能稳定，2例功能恶化。
大型有临床症状的视神经胶质瘤可能会自发消退。NF-1患者和非NF-1患者中均可见消退。消退可能表现为肿瘤大小整体缩小，或磁共振成像上的信号改变。消退可能伴有不同程度的视觉功能改善。在计划治疗这些肿瘤患者时应考虑视神经胶质瘤自发消退的可能性。
