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儿童原发性脊髓胶质瘤:上海一家转诊儿科机构的研究结果。

Primary spinal glioma in children: Results from a referral pediatric institution in Shanghai.

作者信息

Khalil J, Chuanying Z, Qing Z, Belkacémi Y, Mawei J

机构信息

Radiation oncology, National Cancer Institute, Souissi, 10000 Rabat, Morocco.

Radiotherapy, Xhinhua General Hospital, 1665 Kongjiang Road, Yangpu, 10000 Shanghai, China.

出版信息

Cancer Radiother. 2017 Jun;21(4):261-266. doi: 10.1016/j.canrad.2016.11.005. Epub 2017 May 16.

Abstract

PURPOSE

Primary spinal cord glioma is a rare entity especially in children; accounting for less than 10% of all central nervous system tumors. Low grade is the most reported subtype. Treatment modalities have largely evolved; large improvements have been made in the surgical field but also in both of radiotherapy and chemotherapy. Nevertheless, the optimal treatment is yet to be defined.

MATERIAL AND METHODS

A chart review of 11 pediatric patients with a diagnosis of low grade spinal cord glioma at Xhinhua hospital in Shanghai was conducted. A statistical package for Social Sciences Package (SPSS) was used for analysis. Means and standard deviations were calculated. The Kaplan-Meier method was used to analyze overall survival and progression-free survival.

RESULTS

The mean age was 6.7 years (range: 6 months-14.3 years). Revealing symptoms were variable and slowly progressive. The mean duration of symptoms prior to diagnosis was of 7±3.2 months. Astrocytoma was the most commonly reported histological type (seven cases, 63.6%), ependymomas were reported in three cases (27.3%). Surgery was performed in all patients. Subtotal resection concerned the majority of patients (nine patients, 81.8%). Adjuvant radiotherapy was indicated in all cases. A total dose of 39.6Gy was delivered to the whole group. Three patients received adjuvant chemotherapy, of whom two patients had grade III glioma and one patient had a tumor recurrence. Temozolomide-based regimen was the main protocol used for all our patients. The 3 years overall survival rate was 100%, whereas the progression free survival rate was 87.5%. One case relapsed during the next year following completion of treatment.

CONCLUSION

Our preliminary results are consistent with that of other similar published reports, however longer follow up is needed. So are specific recommendations that are still lacking in this setting.

摘要

目的

原发性脊髓胶质瘤是一种罕见疾病,尤其在儿童中;占所有中枢神经系统肿瘤的比例不到10%。低级别是报道最多的亚型。治疗方式有了很大发展;手术领域有了很大改进,放疗和化疗也有改进。然而,最佳治疗方案仍有待确定。

材料与方法

对上海新华医院11例诊断为低级别脊髓胶质瘤的儿科患者进行病历回顾。使用社会科学统计软件包(SPSS)进行分析。计算均值和标准差。采用Kaplan-Meier法分析总生存期和无进展生存期。

结果

平均年龄为6.7岁(范围:6个月至14.3岁)。出现的症状各不相同且进展缓慢。诊断前症状的平均持续时间为7±3.2个月。星形细胞瘤是最常报道的组织学类型(7例,63.6%),室管膜瘤报道了3例(27.3%)。所有患者均接受了手术。大部分患者(9例,81.8%)进行了次全切除。所有病例均进行了辅助放疗。全组放疗总剂量为39.6Gy。3例患者接受了辅助化疗,其中2例为III级胶质瘤,1例为肿瘤复发。替莫唑胺方案是我们所有患者使用的主要方案。3年总生存率为100%,而无进展生存率为87.5%。1例患者在治疗完成后的次年复发。

结论

我们的初步结果与其他类似的已发表报告一致,然而需要更长时间的随访。在这种情况下,具体建议也仍然缺乏。

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