5例颅内伴多层菊形团的胚胎性肿瘤的临床诊疗  

Embryonal tumor with multilayered rosettes:Clinical diagnosis and treatment of 5 cases

作　　者：李曾艳 吴志明 董佑红 王长英 张冬冬 LI Zengyan;WU Zhiming;DONG Youhong;WANG Changying;ZHANG Dongdong(Department of Oncology,Xiangyang No.1 People′s Hospital,Hubei University of Medicine,Xiangyang,441000,China;Department of Orthopedics,Xiangyang No.1 People′s Hospital,Hubei University of Medicine,Xiangyang,441000,China;Department of Pediatric Hematology-Oncology,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200135,China)

机构地区：[1]湖北医药学院附属襄阳市第一人民医院肿瘤科,襄阳441000 [2]湖北医药学院附属襄阳市第一人民医院骨科,襄阳441000 [3]上海交通大学附属新华医院儿童血液肿瘤科,上海200135

出　　处：《中国小儿血液与肿瘤杂志》2023年第5期299-304,共6页Journal of China Pediatric Blood and Cancer

基　　金：湖北医药学院附属襄阳市第一人民医院儿童肿瘤临床医学研究中心(XYY2022P05);襄阳市科技局重点项目(2021YL26);襄阳市第一人民医院博士人才启动金项目(XYY2021D01)。

摘　　要：目的 介绍颅内伴多层菊形团的胚胎性肿瘤(ETMR)的临床特点、治疗方案和临床结局,为该类罕见肿瘤的临床诊疗提供经验。方法 回顾性分析上海交通大学附属新华医院2017年01月—2020年06月收治的ETMR患儿的发病年龄、肿瘤大小、分期、发病部位、治疗方案和预后。结果 本中心4年内共收治5例患儿,其中男孩4例,女孩1例。患儿发病年龄在(16~66)个月,常见临床表现是颅内压增高,5例患儿均行手术+化疗,1例追加辅助放疗。截至随访时间,2例患儿因疾病进展死亡,3例存活。结论 ETMR高度恶性,我国儿童发病年龄较小,诊断需要结合临床表现、影像学及病理组织学检查,以及C19MC基因扩增状态和LIN28蛋白的表达水平。该病早期死亡率高,手术联合化疗是其主要的治疗手段,术后辅助放疗也可使部分患儿受益。Objective This study aimes to share experience for the clinical diagnosis and treatment of intracranial embryonal tumor with multilayered rosettes,ETMR.Methods The age of onset,tumor size,stage,tumor site,treatment regimenand clinical outcome of children with ETMR were retrospectively analyzed.These children were admitted to the Pediatric Hematology Center,Hubei University of Medicine affiliated Xiangyang No.1 Peoples′Hospital between January 2017 and June 2020.Results A total of 5 children were enrolled,including 4 boys and 1 girl.The age of onset of ETMR in children range16 to 66 months.The common clinical presentation was raised intracranial pressure.All the patients were treated with surgery followed by chemotherapy,only one patient received adjuvant radiotherapy.By the end of the follow-up,two patients died due to disease progression,and three patients were alive.Conclusions ETMR is a highly aggressive malignancy with relative low age among Chinese children.The diagnosis of ETMR depends on clinical presentation,imaging and histopathological examination,as well as C19MC gene amplification status and LIN28 protein expression level.ETMR has a very high early mortality,The mainstay treatment for ETMR is surgery combined with chemotherapy,postoperative adjuvant radiotherapy may also benefit some children.

关 键 词：伴多层细胞菊形团的胚胎性肿瘤 手术 辅助化疗 放疗 

分 类 号：R739.41[医药卫生—肿瘤]