摘要
目的:阐明小脑发育不良性节细胞瘤(Lhermitte-Duclos disease,LDD)的临床病理特征。方法:回顾性分析2例小脑发育不良性节细胞瘤的临床表现、组织学和免疫组织化学特点。结果:LDD患者临床主要表现为颅内压增高、共济失调;MRI呈特征性条纹状改变;组织学表现为小脑叶片增大,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。免疫组织化学染色显示肿瘤细胞NSE、Syn、NeuN、CgA阳性,GFAP阴性,Ki-67增殖指数<1%。结论:小脑发育不良性节细胞瘤是一种罕见的良性肿瘤,正确诊断必须依赖于MRI、组织病理学及免疫组织化学标记,首选治疗是肿瘤全切手术,部分病例可伴有Cowden综合征。
Objective： To investigate the clinical and pathological characteristics of dysplastic gangliocytoma of cerebellum （ Lhermitte - Duclos disease, LDD）. Methods： Clinical manifestations, histological and immunohisto- chemical features of 2 LDD patients were analyzed retrospectively. Results： The main clinical manifestations of LDD patients were intracranial hypertension, ataxia. MRI images demonstrated typical striated pattern. The histopathology showed a diffuse enlargement of the cerebellum folium,abnormality of cerebellar parallel layer structure and hyperplasia of molecular layer of cortex. The morphologic abnormal and dysplastic neurons were scattered in the granular cell and Purkinje cell layers of the cerebellar cortex. Immunohistochemical staining showed the tumor cells were positive for NSE, Syn, NeuN, CgA, but negative for GFAP. The Ki - 67 proliferation index was less than 1%. Conclusion： LDD is a rare benign tumor and diagnosed correctly by its characters of MRI, histopathlogy and immunohistochemistry. The first choice of treatment is total tumor resection. Some LDD patients are associated with Cowden syndrome.
出处
《现代肿瘤医学》
CAS
2010年第2期284-286,共3页
Journal of Modern Oncology
