中枢神经细胞瘤的临床病理特征和预后

A clinicopathological and prognostic study of 22 cases central neurocytoma

目的 探讨中枢神经细胞瘤 (CNC)的临床病理特征及其与预后的关系。方法 应用光镜、电镜检查观察了 2 2例CNC的组织病理、超微结构特点 ,应用免疫组织化学染色观察了突触素、神经元特异性烯醇化酶 (NSE)、Leu 7、胶质纤维酸性蛋白 (GFAP)、髓磷脂碱性蛋白 (MBP)及增殖细胞核抗原 (PCNA)等在CNC的表达情况。结果 本组病例年龄 4～ 4 4岁 ,平均 2 7 9岁。肿瘤均发生于脑室。随访 18例 ,14例生存 8个月至 14年 11个月 ;4例死亡 ,平均生存时间 70 7个月。CNC组织学特点 ,瘤细胞有明显的核周空晕 ,呈蜂窝状结构 ,特征性改变为无核纤维岛。细胞异型、核分裂象及灶性坏死罕见。免疫组织化学染色突触素、NSE、Leu 7阳性表达 ,GFAP及MBP阴性反应 ,PCNA极少数细胞阳性。超微结构特点为圆形瘤细胞可见多量细胞突起 ,突起内含微管、神经分泌颗粒、透明囊泡及溶酶体样结构。结论 常规光镜检查难以鉴别CNC和少突胶质细胞瘤 ,免疫组织化学和电镜对确诊CNC很重要。CNC一般预后良好。

Objective To investigate the clinicopathological features and prognosis of 22 cases of central neurocytoma (CNC), representing 0 48% of a series of 4 528 patients undergoing biopsy for central nervous system tumors Methods The histopathological, ultrastructral, immunohistochemical and clinical features of CNC were studied by electron microscopic examination and immunohistochemical stain for Synaptophysin (Syn), neuron special enolase (NSE), Leu 7, glial fibrillary acid protein (GFAP), MBP and proliferating cell nuclear antigen (PCNA) Results The age of the cases ranged from 4 to 44 (average 27 9 years) with all tumors localized in the ventricles In the 18 patients followed up, 14 were alive for 8 months to 14 years and 11 months after the operation, and 4 died The average survival period was 70 7 months Histologically, the tumor in all 22 cases had the oligodendroglioma like pattern with honeycomb appearance and cell free islands of eosinophilic matrix Cellular anaplasia, mitosis and necrotic areas were rarely seen in the tumors Immunohistochemical study demonstrated strong positivity for Syn, NSE and Leu 7, and negative for GFAP and MBP Ultrastructural features showed presence of round tumor cells with abundant cell processes containing microtubules, neurosecretory granules, clear vesicles and lysosome like structures Conclusions The differential diagnosis between CNC and oligodendroglioma could not be established by routine light microscopy The importance of immunohistochemical and electron microscopic studies for making a correct diagnosis is emphasized The prognosis of patients is usually favorable, even if the tumor was resected subtotally The relationship between the presence of anaplastic histological features in CNC and patient outcome remains unclear