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中枢神经细胞瘤:一种人类神经胶质祖细胞瘤的临床、免疫组织化学及生物学特征

Central neurocytoma: clinical, immunohistologic, and biologic findings of a human neuroglial progenitor tumor.

作者信息

Valdueza J M, Westphal M, Vortmeyer A, Muller D, Padberg B, Herrmann H D

机构信息

Department of Neurosurgery, University Hospital Hamburg-Eppendorf, F.R.G.

出版信息

Surg Neurol. 1996 Jan;45(1):49-56. doi: 10.1016/0090-3019(95)00376-2.

DOI:10.1016/0090-3019(95)00376-2
PMID:9190699
Abstract

BACKGROUND

Central neurocytomas are rare brain tumors recognized by their typical radiologic and histologic features. In general, a good prognosis is achieved by total removal. The histogenesis is still under debate, but a neuronal origin is widely assumed.

METHODS

This study presents the clinical and immunohistologic findings of five patients and the results of cell culture experiments of two patients with central neurocytoma treated surgically between 1983 and 1993.

RESULTS

The patient age at diagnosis ranged from 21 to 30 years (mean, 25 years). The male-to-female ration was 1:4. Raised intracranial pressure due to hydrocephalus was the main cause of the clinical manifestations. Total resection was achieved in two cases. Four patients received radiotherapy. One patient suffered a recurrence 1 year after surgery, requiring a second resection and radiotherapy. Follow-up studies took place between 1 and 10.5 years (mean, 7.1 years). To date, all patients are free of their tumors. Two patients suffered from permanent memory disturbances after surgery. Immunohistochemistry confirmed the neuronal nature of the tumors. Cell-culture studies, which have been carried out for the first time, demonstrated concomitant expression of neuronal (synaptophysin) and glial (GFAP) markers.

CONCLUSION

Total removal is the therapy of choice. In tumor recurrence or limited surgery (e.g. due to severe affliction of the fornical structures), radiotherapy has shown to be efficacious. The cell-culture experiments give new insight on the histogenesis of central neurocytoma, indicating that the tumor arises from an undifferentiated precursor cell with the capacity of bipotential neuroglial differentiation.

摘要

背景

中枢神经细胞瘤是一种罕见的脑肿瘤,可通过其典型的放射学和组织学特征得以识别。一般来说,通过全切可获得良好的预后。其组织发生仍存在争议,但普遍认为起源于神经元。

方法

本研究呈现了1983年至1993年间接受手术治疗的5例中枢神经细胞瘤患者的临床和免疫组化结果,以及2例患者的细胞培养实验结果。

结果

确诊时患者年龄在21岁至30岁之间(平均25岁)。男女比例为1:4。脑积水导致的颅内压升高是临床表现的主要原因。2例实现了全切。4例患者接受了放疗。1例患者术后1年复发,需再次切除并放疗。随访时间为1至10.5年(平均7.1年)。迄今为止,所有患者肿瘤均无复发。2例患者术后出现永久性记忆障碍。免疫组化证实了肿瘤的神经元性质。首次进行的细胞培养研究显示神经元(突触素)和神经胶质(胶质纤维酸性蛋白)标志物同时表达。

结论

全切是首选治疗方法。对于肿瘤复发或手术受限(如由于穹窿结构严重受累)的情况,放疗已显示有效。细胞培养实验为中枢神经细胞瘤的组织发生提供了新的见解,表明该肿瘤起源于具有双潜能神经胶质分化能力的未分化前体细胞。

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