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小儿基底神经节区肿瘤:与组织病理学结果相关的临床和放射学特征

Pediatric Basal Ganglia Region Tumors: Clinical and Radiologic Features Correlated with Histopathologic Findings.

作者信息

Fu Wei, Ju Yan, Zhang Si, You Chao

机构信息

Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, Sichuan, China.

Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, Sichuan, China.

出版信息

World Neurosurg. 2017 Jul;103:504-516. doi: 10.1016/j.wneu.2017.04.004. Epub 2017 Apr 10.

Abstract

PURPOSE

To summarize the clinical and radiologic features of pediatric basal ganglia region tumors (PBGRT) in correlation with their histopathologic findings to reduce inappropriate surgery and identify tumors that can benefit from maximal safe resection.

METHODS

The records of 35 children with PBGRT treated in our hospital from December 2011 to December 2015 were analyzed retrospectively. The clinical and radiologic features of these tumors were summarized and correlated with their histopathologic diagnosis.

RESULTS

Our series included 15 astrocytomas and 11 germ cell tumors (GCTs). Basal ganglia astrocytomas were characterized by various clinical presentations and an ill-circumscribed mass with the involvement of surrounding structures on neuroimaging and mostly occurred in the first decade of life (n = 10; 66.7%). Basal ganglia GCT mostly occurred in the second decade of life (n = 8; 72.7%) with hemiparesis as the most common symptom (n = 9; 81.8%). The tumors were located predominantly in the caput of caudate nucleus (n = 8; 72.7%) with hemiatrophy as the typical sign (n = 8; 72.7%). Occasionally, other tumors also could occur in this region, including primitive neuroectodermal tumor (n = 1), atypical teratoid/rhabdoid tumor (n = 1), anaplastic ependymoma (n = 1), lymphoma (n = 1), extraventricular neurocytoma (n = 1), gangliogliomas (n = 2), oligodendroglioma (n = 1), and dysembryoplastic neuroepithelial tumor (n = 1).

CONCLUSIONS

Astrocytoma and GCT are the most common PBGRTs. Low-grade astrocytomas could benefit from maximal surgical resection, whereas GCTs merit neoadjuvant chemoradiation therapy followed by second-look surgery. We advocate routine testing of tumor markers and analysis of their clinical and radiologic features to optimize the therapeutic strategy.

摘要

目的

总结小儿基底节区肿瘤(PBGRT)的临床和放射学特征,并将其与组织病理学结果相关联,以减少不适当的手术,并确定可从最大安全切除中获益的肿瘤。

方法

回顾性分析2011年12月至2015年12月在我院接受治疗的35例PBGRT患儿的病历。总结这些肿瘤的临床和放射学特征,并将其与组织病理学诊断相关联。

结果

我们的系列研究包括15例星形细胞瘤和11例生殖细胞肿瘤(GCT)。基底节区星形细胞瘤的特点是临床表现多样,神经影像学显示肿块边界不清且累及周围结构,大多发生在生命的第一个十年(n = 10;66.7%)。基底节区GCT大多发生在生命的第二个十年(n = 8;72.7%),偏瘫是最常见的症状(n = 9;81.8%)。肿瘤主要位于尾状核头部(n = 8;72.7%),典型征象为半侧萎缩(n = 8;72.7%)。偶尔,该区域也可发生其他肿瘤,包括原始神经外胚层肿瘤(n = 1)、非典型畸胎样/横纹肌样肿瘤(n = 1)、间变性室管膜瘤(n = 1)、淋巴瘤(n = 1)、脑室外神经细胞瘤(n = 1)、神经节胶质瘤(n = 2)、少突胶质细胞瘤(n = 1)和胚胎发育不良性神经上皮肿瘤(n = 1)。

结论

星形细胞瘤和GCT是最常见的PBGRT。低级别星形细胞瘤可从最大程度的手术切除中获益,而GCT则应先进行新辅助放化疗,然后进行二次手术。我们主张常规检测肿瘤标志物并分析其临床和放射学特征,以优化治疗策略。

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