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塞尔维亚 10 年间儿科脑瘤的临床特征、治疗和转归:国家转诊机构的经验。

Clinical profile, treatment and outcome of pediatric brain tumors in Serbia in a 10-year period: A national referral institution experience.

机构信息

Faculty of Medicine, University of Belgrade, Belgrade, Serbia.

Pediatric Radiation Oncology Department, Institute of Oncology and Radiology of Serbia, Belgrade, Serbia.

出版信息

PLoS One. 2021 Oct 26;16(10):e0259095. doi: 10.1371/journal.pone.0259095. eCollection 2021.

Abstract

OBJECTIVE

This study aimed to evaluate the characteristics of children with primary brain tumors, the effectiveness of treatment modalities, and to detect factors related to the outcome.

METHODS

A detailed analysis was performed on a series of 173 pediatric patients treated in a Serbian referral oncology institution between 2007 and 2016, based on their clinical, histological, treatment, and follow-up data.

RESULTS

Mean survival time of all children was 94.5months. 2-, 5- and 10-year overall survival probabilities were 68.8%, 59.4%, and 52.8%, respectively. Patients with supratentorial tumors had longer survival than patients with infratentorial tumors and patients with tumors in both compartments (p = 0.011). Children with the unknown histopathology (brainstem glioma) and high-grade glioma had a shorter life than embryonal tumors, ependymoma, and low-grade glioma (p<0.001). Survival of the children who underwent gross total resection was longer than the children in whom lesser degrees of resection were achieved (p = 0.015). The extent of the disease is a very important parameter found to be associated with survival. Patients with no evidence of disease after surgery had a mean survival of 123 months, compared with 82 months in patients with local residual disease and 55 months in patients with disseminated disease (p<0.001). By the univariate analysis, factors predicting poor outcome in our series were the presentation of disease with hormonal abnormalities, tumor location, and the extent of the disease, while the factors predicting a better outcome were age at the time of diagnosis, presentation of the disease with neurological deficit, and type of resection. By the multivariate analysis, the extent of the disease remained as the only strong adverse risk factor for survival (HR 2.06; 95% CI = 1.38-3.07; p<0.001).

CONCLUSIONS

With an organized and dedicated multidisciplinary team, the adequate outcomes can be achieved in a middle-income country setting. The presence of local residual disease after surgery and disseminated disease has a strong negative effect on survival.

摘要

目的

本研究旨在评估原发性脑肿瘤患儿的特征、治疗方式的疗效,并发现与预后相关的因素。

方法

对 2007 年至 2016 年间在塞尔维亚一家肿瘤转诊机构接受治疗的 173 名儿科患者的临床、组织学、治疗和随访数据进行了详细分析。

结果

所有患儿的平均生存时间为 94.5 个月。2 年、5 年和 10 年的总生存率分别为 68.8%、59.4%和 52.8%。幕上肿瘤患儿的生存时间长于幕下肿瘤患儿和累及两个部位的肿瘤患儿(p = 0.011)。组织学类型为未明确(脑干胶质瘤)和高级别胶质瘤的患儿比胚胎性肿瘤、室管膜瘤和低级别胶质瘤患儿的生存时间短(p<0.001)。行肿瘤全切除的患儿生存时间长于肿瘤部分切除的患儿(p = 0.015)。疾病程度是与生存相关的一个非常重要的参数。术后无疾病证据的患儿平均生存时间为 123 个月,局部残留疾病患儿为 82 个月,播散性疾病患儿为 55 个月(p<0.001)。单因素分析显示,本组患儿预后不良的预测因素包括疾病表现伴有激素异常、肿瘤位置和疾病程度,而预后较好的预测因素包括诊断时的年龄、疾病表现伴有神经功能缺损和切除类型。多因素分析显示,疾病程度仍是影响生存的唯一强不利危险因素(HR 2.06;95%CI = 1.38-3.07;p<0.001)。

结论

在中低收入国家,通过组织化和专业化的多学科团队,可以实现良好的治疗效果。术后局部残留疾病和播散性疾病对生存有强烈的负面影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/210b/8547703/c3f14c2c9692/pone.0259095.g001.jpg

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