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小儿后颅窝室管膜瘤治疗的临床特征及预后因素

Clinical Characteristics and Prognostic Factors of Treatment in Pediatric Posterior Cranial Fossa Ependymoma.

作者信息

Ma Shun-Chang, Li Chun-De, Agazzi Siviero, Jia Wang

机构信息

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.

Department of Neurosurgery, Tampa General Hospital, University of South Florida, Tampa, Florida, USA.

出版信息

Pediatr Neurosurg. 2019;54(2):98-107. doi: 10.1159/000495809. Epub 2019 Jan 30.

DOI:10.1159/000495809
PMID:30699434
Abstract

OBJECTIVE

The purpose of this study was to explore the clinical features and risk factors of outcomes in pediatric posterior cranial fossa ependymoma. We aim to provide evidence-based recommendations for the improvement of prognoses.

PATIENTS AND METHODS

The clinical data, treatment modalities, approaches performed, recurrence rates and times, as well as the outcomes of 94 cases were analyzed retrospectively. The characters of neuroimaging were further studied.

RESULTS

In data from the most recent follow-up, 27 cases had tumor recurrence. The time for tumor recurrence was 13.7 ± 7.7 months. The estimated overall survival and progression-free survival, based on Kaplan-Meier analysis, was 42.2 ± 2.9 months and 38.7 ± 3.4 months, respectively. Univariate analysis showed that being free of recurrence is closely related to the high tumor sphericity (p = 0.018), homogeneity of tumor texture (p = 0.001), and gross total resection (GTR; p < 0.001). Mortality is linked to low sphericity (p = 0.017) and brain stem edema (p = 0.005). Cerebellar mutism is correlated with posterosuperior compression of the 4th ventricle roof by the tumor. The incidence rate of cerebellar ataxia, cerebellar mutism, and cerebellar dysarthria is related to the rostral extension of the tumor within the 4th ventricle. The recurrence rate is higher in subtotal resection (STR) than in GTR, and the difference is significant (p < 0.001). Although there is no significant difference between the recurrence rates in the three types, an earlier recurrence is prone with tumors located in the paramidline-lateral compared to the midline (p = 0.021) and paramidline-medial areas (p = 0.042).

CONCLUSIONS

Based on our data, GTR is indicated as the most optimal choice. Recurrence is linked to lower tumor sphericity, inhomogeneous tumor texture, and STR/partial resection. Tumor located on the lateral side might be prone for an early recurrence.

摘要

目的

本研究旨在探讨小儿后颅窝室管膜瘤的临床特征及预后危险因素。我们旨在为改善预后提供循证建议。

患者与方法

回顾性分析94例患者的临床资料、治疗方式、手术入路、复发率及复发时间,以及预后情况。进一步研究神经影像学特征。

结果

在最近一次随访数据中,27例出现肿瘤复发。肿瘤复发时间为13.7±7.7个月。基于Kaplan-Meier分析,估计总生存期和无进展生存期分别为42.2±2.9个月和38.7±3.4个月。单因素分析显示,无复发与高肿瘤球形度(p = 0.018)、肿瘤质地均匀性(p = 0.001)及全切除(GTR;p < 0.001)密切相关。死亡率与低球形度(p = 0.017)及脑干水肿(p = 0.005)有关。小脑缄默症与肿瘤对第四脑室顶后上部分的压迫有关。小脑共济失调、小脑缄默症和小脑构音障碍的发生率与肿瘤在第四脑室内向头端延伸有关。次全切除(STR)的复发率高于GTR,差异有统计学意义(p < 0.001)。虽然三种类型的复发率之间无显著差异,但与中线(p = 0.021)和中线旁内侧区域(p = 0.042)的肿瘤相比,位于中线旁外侧的肿瘤更容易早期复发。

结论

基于我们的数据,GTR是最理想的选择。复发与较低的肿瘤球形度、不均匀的肿瘤质地以及STR/部分切除有关。位于外侧的肿瘤可能更容易早期复发。

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