小脑发育不良性神经节细胞瘤1例及文献复习  

Lhermitte-Duclos disease:A case report and literature review

作　　者：刘志强 何彦庆 付佳鑫 伍军[1] 宋涛[1] 王莹[1] 黄天翔 LIU Zhiqiang;HE Yanqing;FU Jiaxin;WU Jun;SONG Tao;WANG Ying;HUANG Tianxiang(Department of Neurosurgery,Xiangya Hospital,Central South University,Changsha 410008;Grade of 2015 in Oral Medicine,Medical College of Jinzhou Medical University,Jinzhou Liaoning 121001,China)

机构地区：[1]中南大学湘雅医院神经外科,长沙410008 [2]锦州医科大学医疗学院,辽宁锦州121001

出　　处：《中南大学学报（医学版）》2021年第2期195-199,共5页Journal of Central South University ：Medical Science

基　　金：湖南省自然科学基金(2019JJ40516,2019JJ50964)。

摘　　要：小脑发育不良性神经节细胞瘤(Lhermitte-Duclos disease,LDD)是一种罕见的位于后颅窝的脑部肿瘤。中南大学湘雅医院神经外科收治了1例起源于左侧小脑和蚓部的LDD患者,MRI显示肿瘤在近蚓部区域呈轻度不均匀强化,在2016年8月11日进行肿瘤部分切除后未进行放射治疗和化学治疗。无进展生存时间为11个月,总体生存时间为17个月,说明部分切除不利于患者预后,因为残留的病灶可能会在短期内出现复发。本文还结合相关文献,对LDD的发病原因和诊治方法进行了探讨。Lhermitte-Duclos disease(LDD)is a type of rare brain tumor located in posterior fossa.A patient with LDD located in the left cerebellum and vermis was admitted by the Department of Neurosurgery,Xiangya Hospital,Central South University.MRI scan showed slightly heterogeneous enhancement at the region close to vermis.The patient underwent partial resection on August 11,2016 without postoperative chemoradiotherapy.The progress free survival was 11 months and the overall survival was 17 months.What the case reveals is that the partial resection is not beneficial to these patients with LDD as the residual lesion probably recurs in a short term after operation.The pathogenesis,diagnosis and treatment of LDD are explored and summarized in combination with relevant literature.

关 键 词：小脑发育不良性神经节细胞瘤 诊断 治疗 

分 类 号：R739.41[医药卫生—肿瘤]