53例儿童视路胶质瘤的临床特点及预后分析

Clinical characteristics and treatment of pediatric optic pathway gliomas

目的探讨我国儿童视路胶质瘤的临床特征、治疗方法及预后。方法回顾性分析2003年4月至2011年1月在北京天坛医院小儿神经外科专业病房收治的53例视路胶质瘤患者临床资料，通过随访，分析无进展生存的影响因素。结果本组患者平均年龄7．6岁（10个月～15岁），以DodgeⅡ型为主，均接受肿瘤部分切除术，随访到的41例患者中，其中30例稳定，11例进展，无进展生存期平均为26个月（3—64个月），术后是否接受放疗差异具有统计学意义（P〈0．01），合并神经纤维瘤病1型（NF-1）的2例，占4％。结论我国儿童视路胶质瘤与国外报道的临床特征存在一定差别，肿瘤部分切除术可以有效降低手术并发症，结合术后放射治疗可有效控制肿瘤，获得较好的治疗效果。

Objective To study the clinical characteristics of optic pathway gliomas （OPG）in in China and evaluate the treatment of microsurgical management followed by postoperative radiotherapy for these patients. Methods All OPG cases treated in Beijing Tiantan Hospital from April 2003 to January 2011 were retrospectively reviewed. All patients＇ clinical data were collected and analyzed. Results The mean age of 53 patients at presentation was 7. 6 years （ range 10 months - 15 years） old. There were only 2 cases of NF1 in the OPG patients. Based on the Dodge classification, type I covered 6% （3 cases）, type II 68% （36 cases） and type HI 26% （14 cases）. All tumors were partial resected （ 〈 50%） and postoperative pathology showed fibrillary astrocytes in 27 cases （51%） and pilocytic astrocytomas in 24 （45%）. There were one oligodendrboglioma and one ganglioglioma. 41 patients （77%） were continually followed -up from 3 months to 8 years （31 months in average）. The symptoms were worsen in 11 cases and stable in 30 cases. The mean progression -free survival （PFS） was 26 months （ranged from 3 to 64 months）. The patients received radiotherapy had a significant better PFS than those without radiotherapy （ P 〈 0.01 ）. The radiotherapy side - effects occurred only in 1 patient with hypothyroidism. Conclusions Partial resection of tumor followed by radiotherapy is efficient to control OPG with less complications. There are less NF1 in Han nationality in China compared with OPG patients in existing literatures from other countries.