儿童颅内室管膜瘤的疗效分析

摘要

目的 探讨儿童颅内室管膜瘤的治疗方法以及预后.方法 回顾性分析2012年9月至2016年9月于首都医科大学附属北京天坛医院神经外科首诊治疗的48例儿童颅内室管膜瘤患者的临床资料.48例患儿的肿瘤均为单发,其中肿瘤位于小脑幕下34例、小脑幕上14例.所有患儿均行手术切除,术后予辅助放疗和(或)化疗,并定期进行门诊和电话随访.采用Kaplan-Meier法分析患儿的生存预后,以Cox回归分析探讨患儿生存期的影响因素.结果 48例患者中,31例初次手术时获肿瘤全切除,17例行次全切除.术后病理学结果提示,经典型室管膜瘤[世界卫生组织(WHO)Ⅱ级]4例,间变性室管膜瘤(WHOⅢ级)26例,部分间变性室管膜瘤(介于WHOⅡ、Ⅲ级之间)18例.术后21例仅出现原位复发,其中20例行再次手术;7例发生原位复发合并远处播散,其中4例行再次手术.48例患儿中,术后接受放疗46例次,化疗42例次.2例随访期内因脑积水死亡,14例因肿瘤复发死亡.48例患儿的随访时间为5～68个月,中位时间为34个月.3年无进展生存率和总生存率分别为29.3％和70.8％,28例复发患者的3年总生存率为62.1％.单因素分析结果表明,首次手术切除程度是影响儿童室管膜瘤患者无进展生存期的因素(P=0.027),患者的诊断年龄、肿瘤的发病部位、末次手术切除程度可能为室管膜瘤患者总生存期的影响因素(均P<0.05).多因素分析结果表明,末次手术切除程度(RR=3.568,95％CI:1.638～7.775,P=0.001)为患者总生存期的独立影响因素.结论 对于儿童颅内室管膜瘤患者,手术切除仍为最重要的治疗手段,肿瘤切除程度与生存结局有关.%Objective To investigate the treatment and prognosis of intracranial ependymoma in children. Methods The clinical data of 48 children with intracranial ependymoma who were first diagnosed and treated at Department of Neurosurgery,Beijing Tiantan Hospital,Capital Medical University from September 2012 to September 2016 were retrospectively analyzed. The tumors were solitary in 48 patients, including under-tentorial tumors in 34 cases and supratentorial tumors in 14 cases. All patients underwent surgical resection, adjuvant radiotherapy and/or chemotherapy, and regular outpatient and telephone follow-up. Kaplan-Meier method was used to analyze the survival rate of patients, and Cox regression analysis was used to explore the influencing factors of survival. Results Of the 48 patients, 31 achieved gross total resection at the first operation and 17 received subtotal resection. Postoperative pathological results showed that classical ependymomas ( WHO gradeⅡ) was in 4 cases, anaplastic ependymomas ( WHO gradeⅢ) in 26 cases, and partial anaplastic ependymomas ( between WHO gradeⅡandⅢ) in 18 cases. Postoperative local recurrence occurred in 21 cases, of which 20 cases underwent reoperation. 7 cases developed local recurrence with distant dissemination, of which 4 cases underwent reoperation. Of 48 patients, radiotherapy were performed in 46 cases/times and chemotherapy in 42 patients/times after operation. 2 cases died of hydrocephalus and 14 cases died of recurrence of tumors. The follow-up time of 48 patients ranged from 5 to 68 months, with a median of 34 months. The 3-year progression-free survival rate and overall survival rate were 29. 3％ and 70. 8％respectively. The 3-year overall survival rate of 28 patients with recurrence was 62. 1％. Univariate analysis showed that for children with ependymoma, the degree of first surgical excision was a factor affecting progression free survival(P=0. 027). The age of diagnosis, the location of the tumors and the degree of last surgical excision might be the influencing factors of overall survival in patients with ependymoma ( all P <0. 05). Multivariate analysis showed that the degree of resection (RR=3. 568,95％CI:1. 638-7. 775,P=0. 001) was an independent factor affecting the survival time of patients. Conclusions Surgical resection is still the most important treatment for children with ependymoma. The degree of tumor resection is related to survival outcomes.