骨原始神经外胚层肿瘤的临床及CT与MRI表现  

Clinical Findings and Imaging Features of Osseous Peripheral Primitive Neuroectodermal Tumors

作　　者：李斌[1] 王超[2] 张敏鸣[2] 

机构地区：[1]浙江杭州市余杭区第一人民医院放射科,311100 [2]浙江大学医学院附属第二医院放射科

出　　处：《临床放射学杂志》2015年第9期1448-1452,共5页Journal of Clinical Radiology

摘　　要：目的探讨骨外周性原始神经外胚层肿瘤(p PNET)的临床及影像学表现特点。方法搜集经手术或穿刺病理证实的15例骨p PNET患者的临床、CT及MRI资料,回顾性分析患者的年龄结构、性别特征及肿瘤发病部位和CT(15例)与MRI(11例)表现。结果 15例中男9例,女6例;平均年龄为29岁,其中30岁以下11例。发生在四肢骨7例,椎体和锁骨各2例,肩胛骨、髂骨、上颌骨、下颌骨各1例。CT表现:15例均有周围软组织肿块形成;13例表现为溶骨性的骨质破坏改变;5例可见骨膜反应,其中3例可见明显的日光样骨膜反应。MRI表现:T1WI 11例中8例病变为中等信号,3例为中等偏高信号。T2WI上11例均为不规则中、高混杂信号,病灶边缘不清。增强后10例呈不均匀显著性强化,1例呈中等度强化。免疫组织化学结果:肿瘤均表达CD99,并不同程度地表达神经特异性烯醇酶、波形蛋白,突触素,神经微丝蛋白等,但不表达白细胞共同抗原等。结论骨p PNET以男性稍多见,好发于年青人。CT图像上多表现为溶骨性骨质破坏伴周围软组织肿块形成,偶可见骨膜反应,通常无钙化。MR T1WI呈中等信号,T2WI呈不均匀中高信号为主要表现;增强后呈不规则的显著性强化。骨p PNET影像学表现缺乏特异性,但对了解病变的范围、有无远处转移及制定治疗方案和评价治疗效果会有一定帮助。Objective To analyze clinical and imaging findings of osseous peripheral primitive neuroectodermal tumor（ p PNET）. Methods We retrospectively analyzed the clinical data（ 15 patients）,CT findings（ 15 patients） and MRI findings（ 11 patients） in 15 cases of osseous p PNET being confirmed by histopathological examination. Results Male accounted for 9 cases（ 60%） and female accounted for 6 cases（ 40%）. The average age was 29 years,11 cases were under the age 30（ 73. 3%）. The tumors localized in the extremities bones（ 7 cases（ 46. 67%））,the vertebrae（ 2 cases）,the clavicle（ 2 cases）,scapula（ 1 case）,ilium（ 1 case）,maxilla（ 1 case）,mandible（ 1 case）. CT scan was performed in 15 cases,and CT findings included soft tissue mass（ 15cases）,lytic lesion（ 13cases（ 86. 67 %））,periosteal reaction（ 5 cases（ 33. 33 %））. On T1 WI,soft tissue mass is isointense（ 8 cases（ 72. 73%）） and slightly hyperintense（ 3 cases）. On T2 WI,aggressive soft tissue mass is heterogeneous iso or hyperintensity（ 11cases）. On contrast enhanced T1 WI,marked heterogeneous enhancement was present in 10 cases（ 90. 91%） and intermediate heterogeneous enhancement in 1 cases.Immunohistochemically,tumor cells all showed positive for CD99,partly showed positive for NSE,Vimentin,Syn,NF and negative for LCA. Conclusion Osseous p PNET mainly affects male,and mainly affects adolescents and young adults.The CT findings of osseous p PNET are destructive lesion with a soft tissue mass and occasionally with periosteal reaction.The tumor often demonstrates isodensity with patchy hypodensity areas. Tumor calcification is uncommon. The MRI findings are aggressive soft tissue mass with isointensity on T1 WI and iso or hyperintensity on T2 WI,and the tumor shows marked heterogeneous enhancement after enhancement. Its imaging features are nonspecific,but CT and MRI are useful in delineating the extent of the tumor,identifying distant metastasis,predicting resectability,and moni

关 键 词：骨肿瘤 原始神经外胚层肿瘤 计算机体层成像 磁共振成像 

分 类 号：R738.1[医药卫生—肿瘤]