小脑发育不良性节细胞瘤的病理、影像学特点及治疗  

Pathological,imaging characteristics and treatment of gangliocytoma of dysplastic cerebellum

作　　者：丁江伟 周刚[1] 丁大领[1] 王树凯[1] 陈晨[2] 王凯 DING Jiang-wei;ZHOU Gang;DING Da-ling(Department of Neurosurgery,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450000,China)

机构地区：[1]郑州大学第一附属医院神经外科,郑州450000 [2]郑州大学第一附属医院磁共振室,郑州450000 [3]郑州市人民医院神经外科

出　　处：《临床神经外科杂志》2019年第1期67-71,共5页Journal of Clinical Neurosurgery

基　　金：河南省自然科学基金(162300410311);郑州大学第一附属医院青年创新基金(一附教科[2015]1号)

摘　　要：目的探讨小脑发育不良性节细胞瘤(Lhermitte-Duclos disease,LDD)的病理和影像学特征;以及诊断与鉴别诊断、治疗与预后。方法回顾性分析8例LDD患者的临床资料及组织病理学、免疫组化检查结果;并结合国内外文献进行分析。8例患者均行手术切除肿瘤。结果 MRI表现为病灶多呈T_1WI等低信号相间的条纹状或层状结构,T_2WI为高信号区域内有低信号的条纹状。病理学检查:小脑叶片增大,板层结构异常,分子层增宽,蒲肯野细胞层及颗粒细胞层内散在分布形态异常、发育不良的神经元。免疫组化显示,肿瘤细胞NSE、Syn、NeuN、Cs A阳性,GFAP阳性,Ki-67增殖指数<1。手术肿瘤全切除4例,次全切除3例,部分切除1例;术后症状均改善。结论 LDD的影像学特征为,MRI上病灶呈条纹状或层状结构的"虎斑纹征";病理特点为肿瘤与正常组织呈层状结构,发育不良的神经节细胞取代颗粒细胞,使分子层增厚,浦肯野细胞减少或消失。治疗采用手术切除肿瘤,患者的预后良好,术后无需放化疗。Objective To observe the clinicopathological features,imaging manifestations and immunohistochemistry of the dysplastic gangliocytoma of cerebellum(Lhermitte-Duclos disease,LDD),and to explore the diagnosis and differential diagnosis,treatment and prognosis of the LDD.Methods The clinical manifestations,histological morphology and immunohistochemistry of 8 LDD patients were analyzed retrospectively.Results In imaging studies,T1WI was shown as a striped or layered structure with low signals,and T2WI was characterized by low-signal streaks in the high signal region.Pathological feature of the classic showed that the cerebellum blade increased.The lamellar structure was unusual and the molecular layer was broadening.Purkinje cellular layer and granular cell layer were morphological abnormalities,dysplasia neurons.Immunohistochemistry showed tumor cells NSE,Syn,NeuN,CsA positive.The Ki-67 proliferation index was less than 1%.Conclusion LDD is a rare benign lesion of the clinic.MRI has its relatively characteristic imaging performance,the operation complete resection of the tumor prognosis is good without chemoradiotherapy.

关 键 词：小脑发育不良性节细胞瘤 MRI 病理学 免疫组化 Cowden综合征 

分 类 号：R739.41[医药卫生—肿瘤]