间变性神经节细胞胶质瘤2例临床病理观察及文献复习

Clinicopathological analysis of anaplastic ganglioglioma:report of cases and literature review

目的探讨间变性神经节细胞胶质瘤(anaplastic ganglioglioma,AGG)的临床病理特征、诊断及鉴别诊断。方法回顾性分析2例AGG病人的临床资料,肿瘤均位于额叶。影像学分别考虑血管畸形和恶性胶质瘤,行组织学观察及免疫组织化学分析。结果镜下肿瘤组织由肿瘤性胶质细胞和形态异常神经元构成。后者表现为体积大、突起多、胞质丰富、细胞膜周围Nissl物质聚集,双核或多核以及位置异常等。胶质成分恶性变,分别呈间变性星形细胞瘤和间变性少突胶质细胞瘤样结构。另见钙化、纤细的毛细血管网、血管周围淋巴细胞袖套及嗜酸性小体等。免疫组化结果:形态异常神经元表达神经元核抗原(NeuN)、微管相关蛋白2(MAP-2)、神经丝蛋白(NF)和突触素,胶质背景胶质纤维酸性蛋白(GFAP)和CD34阳性。结论 AGG罕见,在恶性胶质背景中找到典型的神经节细胞胶质瘤结构是其诊断依据。形态学上需要与间变性多形性黄色星形细胞瘤或少突胶质细胞瘤等鉴别。

Objective To investigate the clinicopathological features,diagnosis and differential diagnosis of anaplastic ganglioglioma（AGG）.Methods Clinical data of 2 patients with AGG were analyzed retrospectively,and the tumors were observed by histology and immunohistochemistry.The tumors of 2 patients localized in the frontal lobe and were respectively diagnosed as vascular malformation and malignant glioma by imaging.Results The tumors were composed of neoplastic glial cells and dysmorphic neurons under microscope.Dysmorphic neurons were characterized with large size,many processes,rich cytoplasm,aggregation of perimembranous Nissl substance,presence of bi-or multi-nucleated neurons and abnormal location.Malignant change involved in the glial component, which of the 2 patients showed anaplastic astrocytoma and anaplastic oligodendroglioma respectively.In addition,typical structure of ganglioglioma such as calcifications and slight capillary network,perivascular lymphatic sheath and eosinophilic bodies were frequently identified.Immunohistochemistry results showed that dysplastic neurons were positive for neuro-specific nuclear protein（NeuN）, microtubule-associated protein 2（MAP-2）,neurofilament（NF） and Syn,and glial component was positive for glial fibrillary acidic protein（GFAP） and CD34.Conclusions AGG is rare and its diagnosis mainly depends on finding typical structures of ganglioglioma in the malignant glial background.Differential diagnosis from anaplastic pleomorphic xanthoastrocytoma or oligodendroglioma is recommended.