Natural history and clinical management of optic pathway glioma
Natural history and clinical management of optic pathway glioma
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DOI：
10.1080/02688690310001601216
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发表时间：
2003-08-01
影响因子：
1.1
通讯作者：
Astrup, J
中科院分区：
文献类型：
--
作者：
Astrup, J
对25例连续的视神经胶质瘤（OPG）患者进行了初步观察，如果进展明显，则进行治疗。诊断基于MRI。12例神经纤维瘤病1型（NF 1）患者的OPG疾病自然史较其他患者更为缓慢，通常观察到消退。13名非NF1患者在诊断时肿瘤较大，进展较多。有5个眶内视神经肿瘤（1个进展），19个视交叉肿瘤（12个进展）和1个弥漫性肿瘤。在NF1患者中，OPG在7岁之前出现，在非NF1患者中，也在包括成人在内的老年患者中出现。进行性眶内OPG最好通过手术切除治疗。进行性视交叉肿瘤最好的治疗方法是放射治疗，并通过显著的消退得到良好的反应。例外情况下，外生性视交叉肿瘤可以通过保留视交叉的手术治疗。
Twenty-five consecutive unselected patients with optic pathway glioma (OPG) were primarily observed and treated if progression was evident. Diagnosis was based on MRI. The natural history of the OPG disease was more indolent in 12 patients with neurofibromatosis 1 (NF1) than in the others, and regressions were commonly observed. Thirteen non-NF1 patients had larger tumours at diagnosis and more progressions. There were five intra- orbital optic nerve tumours ( one with progression), 19 chiasmatic tumours ( 12 with progression) and one diffuse tumour. OPG emerges before the age of 7 years in NF1 patients, and in non-NF1 patients also in older patients including adults. Progressive intra- orbital OPGs are best treated by surgical resection. Progressive chiasmatic tumours are best treated by radiotherapy and respond well by marked regression. Exceptionally, exophytic chiasmatic tumours may be treated by chiasm preserving surgery.