儿童侧脑室非典型畸胎瘤样/横纹肌样瘤4例  

Four Cases of Atypical Teratoid/Rhabdoid Tumor of Lateral Ventricles in Children

作　　者：冯佳琪 王馨瑶 包磊[3] 管雯斌[4] 周亚兵[5] 王晓强[1] FENG Jiaqi;WANG Xinyao;BAO Lei;GUAN Wenbin;ZHOU Yabing;WANG Xiaoqiang(Department of Pediatric Neurosurgery,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China;Graduate School,Inner Mongolia Medical University,Hohhot 010100,China;Department of Radiology,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China;Department of Pathology,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China;Department of Traditional Chinese Medicine,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China)

机构地区：[1]上海交通大学医学院附属新华医院小儿神经外科 [2]内蒙古医科大学研究生院,呼和浩特010100 [3]上海交通大学医学院附属新华医院放射科 [4]上海交通大学医学院附属新华医院病理科 [5]上海交通大学医学院附属新华医院中医科,上海200092

出　　处：《协和医学杂志》2024年第3期655-660,共6页Medical Journal of Peking Union Medical College Hospital

基　　金：上海市科技计划(20Y21900500)。

摘　　要：非典型畸胎瘤样/横纹肌样瘤(atypical teratoid/rhabdoid tumor, AT/RT)临床少见且预后不良,多位于幕下或皮层下方,发生于侧脑室较为罕见且预后极差,目前国内仅有6例侧脑室AT/RT相关报道。本文报道4例儿童侧脑室AT/RT患儿的诊疗经过,并通过文献复习对该病的临床表现、鉴别诊断、治疗及预后进行讨论,以提高临床医生对该病的认识,减少漏诊及误诊发生率。Atypical teratoid/ rhabdoid tumor (AT/ RT) is a rare malignancy located primarily in infrat⁃entorial or subcortical areas with a poor prognosis, and rarely in the lateral ventricle with a very poor prognosis.So far, only 6 cases of AT/ RT in lateral ventricle have been reported in China. This article reports the diagnosisand treatment of four children with AT/ RT in the lateral ventricle, and discusses the clinical manifestations,differentiation and diagnosis, treatment and prognosis of the disease through literature review, in order to im⁃prove clinicians understanding of the disease and reduce missed diagnosis and misdiagnosis.

关 键 词：非典型畸胎瘤样/横纹肌样瘤 侧脑室 MRI 治疗 预后 

分 类 号：R72[医药卫生—儿科] R739.41[医药卫生—临床医学]