第四脑室形成菊形团胶质神经元肿瘤临床病理观察.docVIP

第四脑室形成菊形团胶质神经元肿瘤临床病理观察 　　[摘要]目的 探讨第四脑室形成菊形团的胶质神经元肿瘤（RGNT）患者的临床病理特点、影像学改变及鉴别诊断，旨在提高对该病变的认识。方法 回顾性分析2007年11月～2015年7月我院3例RGNT患者的临床、影像及病理学资料，并复习相关文献。结果 患者均为男性，年龄14～26岁，平均19岁，临床表现有眩晕、呕吐、共济失调等，MRI检查示病变均位于第四脑室，T1WI呈稍低信号影、T2WI呈高信号影。组织学上肿瘤具有特征性的双相结构[神经细胞和胶质结构以及小而一致的神经细胞形成菊形团和（或）血管周围假菊形团]，胶质结构绝大部分区域表现为毛细胞型星形细胞瘤的形态。结论 RGNT是一种生长缓慢、罕见的肿瘤，以第四脑室多见，有其独特的组织形态，目前以手术完整切除为主要治疗方法，术后应密切随访 [关键词]脑室肿瘤；胶质神经元肿瘤；第四脑室；菊形团形成；病理特征 [中图分类号] R739.41 [文献标识码] A [文章编号] 1674-4721（2016）12（c）-0011-04 Clinical and pathological observation on the rosette-forming glioneuronal tumor of the fourth ventricle LI Yun-yuan LI Wei-qing MA Xiao-mei XU Rong-rong XIA Chun-yan Department of Pathology，Changzheng Hospital of the Second Military Medical University，Shanghai 200003，China [Abstract]Objective To explore clinical pathological characteristics，imaging changes and differential diagnosis of rosette-forming glioneuronal tumor （RGNT） in the fourth ventricle for improving the understanding of the disease.Methods The clinical，radiological，and pathological features of the three patients with RGNT in our hospital from November 2007 to July 2015 were analyzed retrospectively，and the relevant literature were reviewed.Results All the patients were male with a mean age of 19 years （from 14 to 26）.The clinical symptoms included dizziness，vomiting，ataxia and so on.MRI revealed hypo-intensity on T1-weighted image，hyper-intensity on T2-weighted image.Histopathological findings showed the typical biphasic feature with neurocytic and glial architecture.The neoplastic tissue consisted of small cells with round nuclei resembling neurocytes often arranged to form neurocytic rosettes or perivascular rosettes radiating around central capillaries.The glial component tended to exhibit pilocytic astrocytoma like morphology with long，hair-like processes.Conclusion RGNT in the fourth ventricle is a rare and slow growth neoplasm.The tumor often occur in the fourth ventricle，with its unique histological，immunohistochemical f