Primary cardiac CIC-rearranged undifferentiated sarcoma in an infant

作     者：Meng Zhang Yeran Yang Xiaoxing Guan Xingfeng Yao Yongli Guo Lejian He 

作者机构：Department of PathologyBeijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Beijing Key Laboratory for Pediatric Diseases of OtolaryngologyHead and Neck SurgeryMOE Key Laboratory of Major Diseases in ChildrenBeijing Pediatric Research InstituteBeijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Beijing Advanced Innovation Center for Big Data-Based Precision MedicineBeihang University&Capital Medical UniversityBeijingChina 

出 版 物：《Pediatric Investigation》 (儿科学研究（英文）)

年 卷 期：2021年第5卷第4期

页      面：313-317页

核心收录：

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

基　　金：Special Fund of The Pediatric Medical Coordinated Development Center of Beijing Hospitals Authority[XTCX201806] Beijing Hospitals Authority Clinical medicine Development of Special Funding Support[XMLX202121] Beijing Advanced Innovation Center for Big Data-Based Precision Medicine,Beihang University&Capital Medical University,Beijing[BHME-201904] 

主　　题：Cardiac Sarcoma CIC Child 

摘      要：Introduction:Cardiac neoplasms are particularly rare in children,and the majority of these tumors are *** 10%of cardiac neoplasms are malignant,including soft tissue sarcomas and *** tumors could also be *** EWSR1-negative round or spindle cell undifferentiated sarcoma harboring CIC gene translocation is a highly aggressive malignancy mainly occurring in soft ***,it has not yet been described in the *** presentation:We report a sarcoma that arose from the right ventricle in a 1-year-old ***,it was composed of closely arranged small round or oval undifferentiated cells with fibrovascular separation,hyaline degeneration,and geographical ***,the neoplastic cells exhibited focal membrane positivity for CD99 and diffuse positivity for WT1 and *** in situ hybridization analysis showed EWSR1-negative but CIC-positive split *** breakpoint was also confirmed by whole genome ***:Based on morphological,immunohistochemical and molecular findings,this cardiac mass was diagnosed as CIC-rearranged sarcoma.