中枢神经细胞瘤8例临床诊治分析

Clinicopathological features and surgical treatment in patients with central neurocytoma

目的总结、探讨中枢神经细胞瘤(CN)的临床特征及治疗方法。方法回顾性分析浙江大学医学院附属第一医院2001年1月至2009年12月的8例显微外科手术后病理证实的CN患者的临床表现、影像学特点和手术方法。应用免疫组织化学的EnVision系统进行S100蛋白、Ki-67、突触素(Syn)和胶质纤维酸性蛋白(GFAP)检测。结果 6例肿瘤位于侧脑室内的前中部及室间孔附近,1例位于侧脑室后部,1例位于松果体区。CT表现为中等或稍高密度肿块,其内有多个低密度灶,部分病例见钙化;MRI表现为T1加权成像(T1WI)中等或稍低信号,T2WI不均匀稍高信号,增强后呈轻-中度强化。免疫组织化学结果:S100蛋白1例阳性;Ki-67阳性率为0.3%～5.5%;Syn抗体7例阳性,其中1例弱阳性;GFAP抗体6例阴性,2例少量细胞阳性。全部病例经显微手术治疗,全切除6例,次全切除2例。术后对2例残留的肿瘤进行放射治疗,随访1至4年,效果大多良好,1例复发后再次手术。结论 CN具有一定特征性的影像学表现。免疫组织化学Syn呈高表达及GFAP和S100蛋白呈低表达对CN的诊断和鉴别诊断具有重要价值。显微外科手术切除并对残余肿瘤放射治疗,可取得良好预后。

Objective To investigate the pathological characteristics and the treatment of central neuroeytoma （CN）. Methods Eight patients with CN, together with clinical manifestations, imaging features, and surgical methods, from January 2001 to December 2009 were retrospectively analyzed. The S100 protein, Ki-67, synaptophysin （Syn） and glial fibrillary acidic protein （GFAP） were studied using envision system immunohistochemistry. Results There were 6 cases whose tumors located in the central and anterior part of lateral ventricle, 1 in the posterior part of lateral ventricle, and 1 in the pineal region. Their CT images showed equal or slightly high density mass in which there were multiple small low density area and calcification. MR images showed equal or slightly low signal in T1 weighted imaging （T1WI）, uneven and slightly high signal in T2WI, and mild to moderate signal after enhancement. Immunohistochemical results showed 7 cases were positive in Syn, 1 was positive in S100, 6 were negative and 2 were partly positive in GFAP. The positive rate of Ki-67 was 0.3%- 5.5%. All patients were treated with microsurgery. Total resections were taken in 6 cases, subtotal resections were taken in 2 cases and postoperative radiotherapy was given to residual tumor. All the cases were followed up from 1 to 4 years, and the results showed only 1 case developed a recurrent tumor and received a total resection again, and the others got good curative effect. Conclusions CN manifests certain characteristic imaging features. High expression of Syn, low expression of GFAP and S100 in CN are valuable for the diagnosis and differentiation. The treatment of microsurgical resection and radiotherapy to residual tumor can achieve good curative effect.