婴幼儿、青少年及成人非典型畸胎样/横纹肌样肉瘤影像表现  

Imaging findings of atypical terratoid/rhabdoid tumors in infant,teenager and adult

作　　者：张玉艳 韩雯雯 牛春艳 倪立桐 齐琦 曹豆豆 李世俊 ZHANG Yuyan;HAN Wenwen;NIU Chunyan;NI Litong;QI Qi;CAO Doudou;LI Shijun(Department of Medical Imaging,Dongying People's Hospital(Dongying Hospital of Shangdong Provincial Hospital Group),Dongying 257091,China;Department of Radiology,the First Medical Center,Chinese PLA General Hospital,Beijing 100853,China)

机构地区：[1]山东省东营市人民医院(山东省立医院集团东营医院)影像科,山东东营257091 [2]中国人民解放军总医院第一医学中心放射诊断科,北京100853

出　　处：《医学影像学杂志》2025年第3期11-15,共5页Journal of Medical Imaging

摘　　要：目的 探讨不同年龄阶段,婴幼儿、青少年及成人原发性颅内非典型畸胎样/横纹肌样肉瘤(atypical teratoid tumors,AT/RT)患者影像表现、病理特征及鉴别诊断,提高对该病的认识及临床诊断符合率。方法 选取并分析5例经临床、影像学检查及手术病理确诊的AT/RT患者资料。其中行CT检查1例,MRI检查3例,CT及MRI检查1例。结果 婴幼儿3例(女性1例,男性2例,平均年龄32个月),青少年1例(男性,12岁),成人1例(女性,28岁)。病变体积大,可累及幕上或幕下,也可幕上及幕下均累及,成人也可见幕下。病变影像表现为混杂密度/信号,内可见多发钙化及位于边缘的少许囊变及出血;SWI显示婴幼儿出血为著,伴少许钙化,青少年则相反;MRS显示Cho峰及Lac峰升高,Naa峰降低;ASL显示婴幼儿部分高灌注,青少年低及中等灌注。结论 AT/RT多见于婴幼儿,青少年及成人罕见,婴幼儿大多位于幕下,也可幕上及幕下均累及,成人一般位于幕上,也可位于幕下。病变体积较大,密度/信号混杂,囊变及出血位于肿瘤边缘具有一定提示特点,但确诊仍需依靠组织病理学检查。Objective To explore the imaging manifestations,pathological features and differential diagnosis of patients with primary intracranial atypical teratoid/rhabdomyosarcoma(AT/RT)at different ages,including infants,adolescents and adults,so as to improve the understanding of the disease and the clinical diagnostic compliance rate.Methods The clinical,imaging and case data confirmed by surgical pathology of five patients with AT/RT were retrospectively analyzed.Among them,one case underwent CT,three cases underwent MRI,and one case was subjected to CT and MRI.Results 3 cases were infants and chil-dren(1 female,2 males,with a mean age of 32 months),and 1 case was adolescent(male,age 12)and 1 case was adult(fe-male,age 28).The lesion was large and may be in supratentorial/subtentorial or both supratentorial and subtentorial areas,and the subtentorial area was also seen in adults;the image showed mixed density/signal,with multiple calcifications and few cystic changes and hemorrhages located at the margins;SWI bleeding in infants and children was prominent with little calcification,while the opposite was true in adolescents;MRS showed elevated Cho and Lac peaks and diminished Naa peaks;ASL showed partial hyperperfusion in infants and young children and low and moderate perfusion in adolescents.Conclusion AT/RT is most common in infants and young children,and rare in adolescents and adults.In infants and young children,the tumor is mostly located in the infratentorial area,or both supratentorial and infratentorial areas,while in adults,the tumor is usually in supratentorial or infratentorial area.The large size of the lesion,mixed density/signal,cystic degeneration and hemorrhage at the edge of the tumor are suggestive features,but diagnosis still needs to rely on histopathology.

关 键 词：非畸胎瘤样/横纹肌样肉瘤 体层摄影术 X线计算机 磁共振成像 

分 类 号：R739.41[医药卫生—肿瘤] R445[医药卫生—临床医学]