不同分子亚型髓母细胞瘤的影像表现及预后分析  

Imaging and prognostic analysis in different molecular subtypes medulloblastoma

作　　者：张雨婷[1] 李禄生[2] 梁平[2] 翟瑄[2] 何玲[1] 蔡金华[1] ZHANG Yuting;LI Lusheng;LIANG Ping;ZHAI Xuan;HE Ling;CAI Jinhua(Department of Radiology, Children’s Hospital, Chongqing Medical University, and Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing International Science and Technology Cooperation Center for Child Development and Disorders and Key Laboratory of Pediatrics in Chongqing;Department of Neurosurgery, Pediatric Surgery （National Key Clinical Specialist）, Children’s Hospital, Chongqing Medical University, Chongqing 400014, China)

机构地区：[1]重庆医科大学附属儿童医院放射科儿童发育疾病研究教育部重点实验室儿童发育重大疾病国家国际科技合作基地儿科学重庆市重点实验室 [2]重庆医科大学附属儿童医院小儿外科国家重点临床专科神经外科,重庆400014

出　　处：《临床儿科杂志》2018年第5期334-338,共5页Journal of Clinical Pediatrics

基　　金：重庆市卫生计生委医学科研项目(No.2015MSXM044);重庆市卫生计生委后备高端医学人才项目(No.2017HBRC007)

摘　　要：目的探讨髓母细胞瘤的分子亚型,分析分子亚型与预后的相关性。方法收集2010—2013年经手术治疗的原发髓母细胞瘤患儿32例,以免疫组化方法检测手术标本中GAB1和YAP1蛋白并确定分子亚型。分析不同分子亚型的临床特点、影像表现及生存情况,比较分子亚型与预后的相关性。结果 32例患儿中男21例、女11例,其中SHH型、WNT型和非SHH/WNT型分别为4例、7例和21例。不同分子亚型患儿的年龄、性别、病理分型、髓母细胞瘤发生位置、T 2信号、DWI信号、强化形式、有无囊变、播散的差异均无统计学意义(P>0.05)。SHH型、WNT型和非SHH/WNT型的3年无事件生存率分别为75%、57.1%、38.1%,差异无统计学意义(P>0.05);≤3岁组非SHH/WNT型的3年无事件生存率为80%,>3岁组为25%,差异有统计学意义(P=0.047)。结论不同分子亚型髓母细胞瘤的预后有SHH型优于WNT型,WNT型优于非SHH/WNT型的趋势;≤3岁非SHH/WNT型的预后好于>3岁者。Objective To detect molecular subtypes of medulloblastoma, and its correlation with prognosis. Methods Surgically treated 32 cases of primary medulloblastoma from 2010-2013 were collected, the molecular subtypes were determined by immunohistochemical detection of GAB1 and YAP1 protein in the sample. Clinical characteristics, imaging features and survival condition of different molecular subtypes were analyzed. Results Molecular typing of the 32 cases（21 males and 11 females） shows 4（12.5%） cases of SHH, 7（21.9%） cases of WNT and 21（65.6%） cases of non-SHH. There was no significant correlation of molecular subtypes with age, gender and pathological classification. Three-year progression free survival rate in SHH, WNT and non-SHH/WNT subtypes were 75%, 57.1% and 38.1%, respectively. Three-year progression free survival rate was significantly higher in patients under 3 years old group than that in patients over 3 years old group in non SHH/WNT（P=0.047）. Conclusions The prognosis of SHH was better than WNT, WNT was better than non-SHH/WNT type, prognosis in patients under 3 years old group of non-SHH/WNT was better than that in patients over 3 years old group.

关 键 词：髓母细胞瘤 分子亚型 3年无事件生存率 儿童 

分 类 号：R739.4[医药卫生—肿瘤]