目的：探讨颅内非典型畸胎瘤样/横纹肌样瘤（AT/RT）的影像表现特征。资料与方法：对2013年11月—2017年1月6例经病理和免疫组化诊断为AT/RT患者的临床及MRI（n=6）影像资料进行回顾性分析。男5例，女1例，年龄1~7岁，中位年龄1.25岁。6例均行MR平扫及增强检查。结果：6例AT/RT患者中4例肿瘤位于幕下，1例位于幕上，1例幕上及幕下多发病灶，肿瘤最大径为2.0~4.6 cm，6例肿瘤合并囊变5例、出血4例，所有病灶都呈中重度强化。DWI呈不均匀高信号，ADC呈低信号。其中有1例发生脑脊液播散，1例术后出现脑转移。结论：颅内AT/RT具有一定特征性的影像学表现，有助于鉴别诊断。
Abstract
Objective: To evaluate the imaging characteristics of intracranial atypical teratoid/rhabdoid tumor(AT/RT), and to improve the diagnostic ability of the disease. Materials and Methods: The clinical and MRI(n=6) imaging findings of 6 patients with AT/RT diagnosed by pathology and immunohistochemistry were analyzed retrospectively. There were 5 male and 1 female, aged 1 year to 7 years, median age was 1.25 years. MR enhancement studies were obtained in all the cases. Results: Among 6 patients with AT/RT, 4 were located below the tentorium, 1 was located supratentorial, and 1 was multiple lesions both supratentorial and subtentorial. The tumors’ size varied from 2.0 cm to 4.6 cm. Cystic change was seen in 5 cases and hemorrhage in 4 cases. All lesions showed moderate to severe enhancement. Diffused-weighted imaging demonstrated inhomogeneous signal and ADC showed low signal. Cerebrospinal fluid dissemination occurred in 1 case, and brain metastasis occurred in 1 case. Conclusion: There are some relatively specific imaging findings of primary intracranial AT/RT that could assist their diagnosis.
关键词
脑肿瘤 /
肿瘤 /
生殖细胞和胚胎性 /
儿童 /
磁共振成像
Key words
Brain neoplasms /
Neoplasms, germ cell and embryonal /
Child /
Magnetic resonance imaging
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参考文献
[1]Jin B, Feng XY. MRI features of atypical teratoid/rhabdoid tumors in children[J]. Pediatr Radiol, 2013, 43(8): 1001-1008.
[2]Fenton LZ, Foreman NK. Atypical teratoid/rhabdoid tumor of the central nervous system in children: an atypical series and review[J]. Pediatr Radoil, 2003, 33(8): 554-558.
[3]Warmuth-Metz M, Bison B, Dannemann-Sterm E, et al. CT and MR imaging in atypical teratoid/rhabdoid tumors of the central nervous system[J]. Neuroradiology, 2008, 50(5): 447-452.
[4]Han L, Qiu Y, Xie C, et al. Atypical teratoid/rhabdoid tumors in adult patient: CT and MR imaging features[J]. AJNR, 2011, 32(1): 103-108.
[5]施伟，李昊，赵瑞. 婴幼儿中枢神经系统非典型畸胎样/横纹肌样瘤的治疗体会[J]. 中华神经外科杂志，2016，32（4）：349-352.
[6]de León-Bojorge B, Rueda-Franco F, Anaya-Jara M. Central nervous system atypical teratoid rhabdoid tumor: experience at the National Institute of Pediatrics, Mexico City[J]. Childs Nerv Syst, 2008, 24(3): 307-312.
[7]Lee IH, Yoo SY, Kim JH, et al. Atypical teratoid tumors of the central nervous system: imaging and clinical findings in 16 children[J]. Clin Radiol, 2009, 64(3): 256-264.
[8]朱华晨，周剑，王志平，等. 中枢神经系统非典型畸胎样/横纹肌样瘤的影像与病理学研究[J]. 实用放射学，2014，30（8）：1263-1266.
[9]沈文婷，沈全力，乔中伟. 颅内非典型畸胎瘤样/横纹肌样瘤的影像学表现[J]. 放射学实践，2015，30（6）：638-642.
[10]Cheng YC, Lirng JF, Chang FC, et al. Neuroradiological findings in atypical teratoid/rhabdoid tumor of the central nervous system[J]. Acta Radiol, 2005, 46(1): 89-96.
[11]Meyers SP, Khademian ZP, Biegel JA, et al. Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes[J]. AJNR, 2006, 27(5): 962-971.
[12]Tez S, Koktener A, Güler G, et al. Atypical teratoid/rhabdoid tumors: imaging findings of two cases and review of the literature[J]. Turk Neurosurg, 2008, 18(1): 30-34.
[13]Arslanoglu A, Aygun N, Tekhtani D, et al. Imaging findings of CNS atypical teratoid/rhabdoid tumors[J]. AJNR, 2004, 25(3): 476-480.
[14]Koral K, Gargan L, Bowers DC, et al. Imaging characteristics of atypical teratoid-rhabdoid tumor in children compared with medulloblastoma[J]. AJR, 2008, 190(3): 809-814.
[15]Schrey D, Carceller LF, Malietzis G, et al. Multimodal therapy in children and adolescents with newly diagnosed atypical teratoid rhabdoid tumor: individual pooled data analysis and review of the literature[J]. J Neurooncol, 2016, 126(1): 81-90.
