INTRACRANIAL EPENDYMOMAS OF CHILDHOOD - LONG-TERM OUTCOME AND PROGNOSTIC FACTORS
INTRACRANIAL EPENDYMOMAS OF CHILDHOOD - LONG-TERM OUTCOME AND PROGNOSTIC FACTORS
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DOI：
10.1227/00006123-199510000-00008
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发表时间：
1995-10-01
期刊：
影响因子：
4.8
通讯作者：
DEUTSCH, M
中科院分区：
文献类型：
--
作者：
POLLACK, IF;GERSZTEN, PC;DEUTSCH, M
我们对1975年至1993年间在我们机构治疗的40例颅内室管膜瘤患儿进行了详细的结局分析，以确定预测总体生存率和无进展生存率的因素。在研究的前5年接受治疗的3名患者（7.5%）在手术后3个月内死亡，并被排除在进一步的结局评估之外。根据影像学（3例儿童）和/或细胞学（6例儿童）结果，在围手术期存活的37例患者中有8例（22%）在就诊时有软脑膜播散的证据。这37例患者的5年和10年无进展生存率分别为45.1%和36.1%;总生存率分别为57.1%和45.0%。19例疾病进展患者中有17例的进展部位为局部。在单变量和多变量分析中，发现三个因素与结果有显著相关性（P <或等于0.05）：1）切除范围，2）诊断时患者的年龄，3）诊断前症状的持续时间。在术后影像学检查有残留病灶证据的患者中，5年无进展生存率和总生存率分别为8.9%和22%，而在无明显残留病灶的患者中，5年无进展生存率和总生存率分别为68%和80%（分别为P = 0.0001和P < 0.0001）。年龄小于3岁的患者的情况明显差于年龄较大的儿童（5年无进展生存率和总生存率分别为12%和22%，而年龄较大的儿童为60%和75%（分别为P = 0.003和P = 0.01））。此外，诊断前症状持续时间较长的患者，
A DETAILED OUTCOME analysis was performed on 40 children with intracranial ependymomas treated at our institution between 1975 and 1993 to identify those factors that were predictive of overall and progression-free survival. Three patients (7.5%) who were treated in the first 5 years of the study died within 3 months of surgery and were excluded from further outcome assessments. Eight (22%) of the 37 patients who survived the perioperative period had evidence of leptomeningeal dissemination at presentation, on the basis of either imaging (three children) and/or cytological (six children) results. The 5- and 10-year progression-free survival rates among these 37 patients were 45.1 and 36.1%, respectively; overall survival rates were 57.1 and 45.0%, respectively. The site of progression was local in 17 of 19 patients with progressive disease. Three factors were found to have a significant association (P less than or equal to 0.05) with the outcome on both univariate and multivariate analyses: 1) the extent of the resection, 2) the age of the patient at diagnosis, and 3) the duration of the symptoms before diagnosis. The 5-year progression-free and overall survivals were 8.9 and 22%, respectively, among patients who had evidence of residual disease on postoperative imaging studies, compared with 68 and 80% rates among patients with no apparent residual disease (P = 0.0001 and P < 0.0001, respectively). Patients younger than 3 years fared significantly worse than older children (5-year progression-free and overall survival rates of 12 and 22%, respectively, in the younger children versus 60 and 75% in older children (P = 0.003 and P = 0.01, respectively). In addition, patients with a duration of symptoms before diagnosis of