临床神经外科杂志2019，Vol.16Issue(1)：67-71,5.DOI:10.3969/j.issn.1672-7770.2019.01.015
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
摘要
Abstract
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, Neu N, Cs A 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Key words
Lhermitte-Duclos disease/MRI/pathology/immunohistochemistry/cowden syndrome分类
医药卫生引用本文复制引用
丁江伟,周刚,丁大领,王树凯,陈晨,王凯..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):67-71,5.基金项目
河南省自然科学基金 (162300410311) （162300410311）
郑州大学第一附属医院青年创新基金 (一附教科[2015]1号) （一附教科[2015]1号）
