[1]丁江伟,周刚,丁大领,等.小脑发育不良性节细胞瘤的病理、影像学特点及治疗[J].临床神经外科杂志,2019,16(1):67-71.[doi:10.3969/j.issn.1672-7770.2019.01.015]
 DING Jiang-wei,ZHOU Gang,DING Da-ling,et al.Pathological, imaging characteristics and treatment of gangliocytoma of dysplastic cerebellum[J].Journal of Clinical Neurosurgery,2019,16(1):67-71.[doi:10.3969/j.issn.1672-7770.2019.01.015]
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小脑发育不良性节细胞瘤的病理、影像学特点及治疗()
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《临床神经外科杂志》[ISSN:1672-7770/CN:32-1727/R]

卷:
16
期数:
2019年第1期
页码:
67-71
栏目:
临床研究
出版日期:
2019-02-18

文章信息/Info

Title:
Pathological, imaging characteristics and treatment of gangliocytoma of dysplastic cerebellum
作者:
丁江伟周刚丁大领王树凯陈晨王凯
450000 郑州,郑州大学第一附属医院神经外科(丁江伟,周刚,丁大领,王树凯),磁共振室(陈晨);郑州市人民医院神经外科(王凯)
Author(s):
DING Jiang-wei ZHOU Gang DING Da-ling et al.
Department of Neurosurgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450000, China
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
Keywords:
Lhermitte-Duclos disease MRI pathology immunohistochemistry cowden syndrome
分类号:
R739.41
DOI:
10.3969/j.issn.1672-7770.2019.01.015
文献标志码:
D
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T1WI等低信号相间的条纹状或层状结构,T2WI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、CsA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。
Abstract:
Abstract: ObjectiveTo 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. MethodsThe clinical manifestations, histological morphology and immunohistochemistry of 8 LDD patients were analyzed retrospectively. ResultsIn 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%. ConclusionLDD 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.

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更新日期/Last Update: 2019-02-18