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伴发颅内肿瘤的Maffucci综合征二例报告及文献复习
引用本文:郝淑煜,张俊廷,耿素民,于兰冰,辛宇,王亮,吴震,张力伟,王忠诚.伴发颅内肿瘤的Maffucci综合征二例报告及文献复习[J].中华神经外科杂志,2009,25(11).
作者姓名:郝淑煜  张俊廷  耿素民  于兰冰  辛宇  王亮  吴震  张力伟  王忠诚
作者单位:1. 首都医科大学北京市神经外科研究所,100050
2. 首都医科大学附属北京天坛医院神经外科中心
基金项目:首都医学发展科研基金 
摘    要:目的 报告2例伴有颅内病变的Maffucci综合征病例并文献回顾.方法 通过全身体检进行诊断,进行手术治疗切除颅内病变.结果 结合多发海绵状血管瘤和软骨瘤等全身表现,符合Maffucci综合征临床诊断.结论 我们首次报告了中国人群中伴有颅内病变的Maffucci综合征病例,对Maffucci综合征相关基因及临床表现仍待进一步研究.

关 键 词:海绵状血管瘤  软骨瘤  Maffucci综合征

Intracranial tumors arising in Maffucci syndrome: two cases report and literature review
HAO Shu-yu,ZHANG Jun-ting,GENG Su-min,YU Lan-bing,XIN Yu,WANG Liang,WU Zhen,ZHANG Li-wei,WANG Zhong-cheng.Intracranial tumors arising in Maffucci syndrome: two cases report and literature review[J].Chinese Journal of Neurosurgery,2009,25(11).
Authors:HAO Shu-yu  ZHANG Jun-ting  GENG Su-min  YU Lan-bing  XIN Yu  WANG Liang  WU Zhen  ZHANG Li-wei  WANG Zhong-cheng
Abstract:Objective To report two cases of Maffucci syndrome with intracranial tumors, and review the correlative literatures. Methods The diagnosis was conformed by physical examination, X-ray, CT and MRI. Operations were performed for intracranial lesions of the two patients. Results According to their clinical symptom and sign,they were diagnosed as Maffucci syndrome. Conclusions This is the first cases of Maffucci syndrome with intracranial tumors in China,we should emphasize further study of the associated genes of Maffucci syndrome and clinical manifestation.
Keywords:Angiocavernoma  Chondroma  Maffucci syndrome
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