Optic Pathway Gliomas: Clinical Manifestation, Treatment, and Follow-Up
Optic Pathway Gliomas: Clinical Manifestation, Treatment, and Follow-Up
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DOI：
10.1159/000445064
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发表时间：
2016-01-01
影响因子：
0.7
通讯作者：
Nejat, Farideh
中科院分区：
文献类型：
--
作者：
Ertiaei, Abolhasan;Hanaei, Sara;Nejat, Farideh
目的：视神经通路胶质瘤（OPG）是一种少见的儿童脑肿瘤，主要影响大脑视神经和视觉通路。不同阶段可能出现多种体征和症状，如视力损害、眼球震颤、眼球突出和视力丧失。其他表现，如脑积水、双头综合征、神经功能缺损、生长发育迟缓，也可能出现。手术切除、化疗和放疗是治疗策略。本研究的目的是评估OPG患者的临床表现及其对治疗的最终结果。方法：对37例初次诊断为OPG的患者进行了为期10年的临床表现、治疗和治疗反应的研究，作为单中心回顾性队列的一部分。结果：患者平均年龄37.1个月，性别分布基本均匀。最常见的表现为视力障碍（94.4%），其他症状包括眼球震颤（50%）、生长发育迟缓（27.8%）、神经功能障碍（19.4%）、间脑综合征（13.5%）、突出（11.1%）和脑积水（29.7%）。肿瘤分期显示10.8%的病例为A期，54.1%为B期，35.1%为c期。在30例可评估的患者中，对治疗的反应包括完全缓解（CR; 23.3%），部分缓解（PR; 43.3%），疾病稳定（23.3%），疾病进展（3.3%）和疾病死亡（6.7%）。肿瘤分期与治疗反应之间存在显著相关性，B期CR和PR较高，C期死亡率较高（p = 0.005）。结论：OPG多见于10岁前，以视觉障碍为最常见的症状。肿瘤分期和治疗策略会影响对治疗的反应，但也应考虑每个患者的特定肿瘤行为。(C) 2016 S. Karger AG，巴塞尔
Objective: Optic pathway gliomas (OPG) are infrequent pediatric brain tumors that affect the optic nerve and the visual pathway in the brain. A wide spectrum of signs and symptoms, such as visual impairment, nystagmus, proptosis, and visual loss, may occur at different stages. Other manifestations, such as hydrocephalus, diancephalic syndrome, neurologic deficits, and growth and developmental delays, may be present as well. Surgical resection, chemotherapy, and radiotherapy are used as treatment strategies. The purpose of this study is to evaluate the clinical presentation of OPG patients and their final outcomes in response to treatment. Methods: Thirty-seven patients with an initial diagnosis of OPG were studied as part of a single-center retrospective cohort for their clinical presentation, treatment, and response to treatment over a 10-year period. Results: The mean age of the patients was 37.1 months, and there was a nearly equal sex distribution. The most prevalent manifestation was visual impairment (94.4%), and other symptoms included nystagmus (50%), growth and developmental delays (27.8%), neurological deficits (19.4%), diencephalic syndrome (13.5%), proptosis (11.1%), and hydrocephalus (29.7%). Tumor staging revealed that 10.8% of the cases were stage A, 54.1% were stage B, and 35.1% were stage C. In 30 evaluable patients, the response to treatment included complete response (CR; 23.3%), partial response (PR; 43.3%), stable disease (23.3%), progressive disease (3.3%), and death due to disease (6.7%). A significant association was detected between tumor staging and the response to treatment, with higher rates of CR and PR in stage B and a higher mortality rate in stage C (p = 0.005). Conclusion: OPG occur more commonly in the first decade of life, with visual disturbance as the most prevalent symptom. Tumor staging and the treatment strategy are proposed to affect the response to treatment, although the specific tumor behavior in each patient should be considered as well. (C) 2016 S. Karger AG, Basel