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儿童基底节区生殖细胞肿瘤的临床与影像学特征

Clinical and Radiologic Features of Pediatric Basal Ganglia Germ Cell Tumors.

作者信息

Zhang Si, Liang Guopeng, Ju Yan, You Chao

机构信息

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

Intensive Care Unit, West China Hospital of Sichuan University, Chengdu, Sichuan, China.

出版信息

World Neurosurg. 2016 Nov;95:516-524.e1. doi: 10.1016/j.wneu.2016.08.072. Epub 2016 Aug 24.

Abstract

BACKGROUND AND OBJECTIVE

Pediatric basal ganglia germ cell tumors (GCTs) represent a rare subset of tumors about which little is known. We aimed to summarize the clinical features and radiological findings of this special subgroup of GCTs.

METHODS

From January 2010 to January 2015, 12 pediatric patients with basal ganglia GCTs were treated in our hospital. The clinical features, radiologic findings, diagnosis, treatment, and outcome of these patients were analyzed retrospectively. Our institutional diagnostic principle and treatment strategy of this disease were discussed.

RESULTS

GCTs accounted for 25.5% of all the pediatric basal ganglia tumors treated in our hospital. There were 9 male and 3 female patients with a mean age of 11.5 ± 2.1 years. The most common symptom was progressive hemiparesis (n = 9, 75%). The radiologic findings showed that the lesions predominately located in caput of caudate nucleus (n = 9, 75.0%), followed by lenticular nucleus (n = 3, 25.0%). Hemiatrophy was commonly observed (n = 8, 66.7%). Eight patients were diagnosed as having germinomas, and 4 patients as having nongerminomatous germ cell tumors. During the follow-up period, preoperative neurologic dysfunctions improved in 7 patients and remained stable in 3. Two patients developed new onset of neurologic dysfunction after the treatment. Two patients suffered from tumor recurrence.

CONCLUSIONS

GCTs are not as rare as considered in pediatric basal ganglia tumors. They bear some distinctive clinical and radiologic features, which can help with the accurate diagnosis and successful management of such tumors.

摘要

背景与目的

小儿基底节区生殖细胞肿瘤(GCTs)是一种罕见的肿瘤类型,目前对其了解甚少。我们旨在总结这一特殊亚组GCTs的临床特征和影像学表现。

方法

回顾性分析2010年1月至2015年1月在我院接受治疗的12例小儿基底节区GCTs患者的临床特征、影像学表现、诊断、治疗及预后情况。并讨论了我院对该疾病的诊断原则和治疗策略。

结果

GCTs占我院治疗的所有小儿基底节区肿瘤的25.5%。其中男性9例,女性3例,平均年龄11.5±2.1岁。最常见的症状是进行性偏瘫(n = 9,75%)。影像学表现显示病变主要位于尾状核头部(n = 9,75.0%),其次为豆状核(n = 3,25.0%)。常见半侧萎缩(n = 8,66.7%)。8例患者诊断为生殖细胞瘤,4例为非生殖细胞性生殖细胞肿瘤。随访期间,7例患者术前神经功能障碍有所改善,3例保持稳定。2例患者治疗后出现新发神经功能障碍。2例患者出现肿瘤复发。

结论

GCTs在小儿基底节区肿瘤中并非如人们认为的那样罕见。它们具有一些独特临床和影像学特征,有助于对此类肿瘤进行准确诊断和成功治疗。

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