骨原发假肌源性(上皮样肉瘤样)血管内皮瘤的临床病理及影像学特征  

Imaging and clinicopathological characteristics of primary skeletal pseudomyogenic(epithelioid sareoma-like)haemangioendothelioma

作　　者：衣利磊[1] 刘壮盛 谢乐[1] 周守国[1] 张家雄[1] 王娟[1] YI Li-lei;LIU Zhuang-sheng;XIE Le(Department of Radiology,the Affiliated Foshan Hospital of Traditional Chinese Medicine,Guangzhou University of Traditional Chinese Medicine,Guangdong 528000,China)

机构地区：[1]广州中医药大学附属佛山市中医院放射科,广东528000 [2]中山大学附属江门医院放射科,广东529030

出　　处：《放射学实践》2018年第4期417-422,共6页Radiologic Practice

基　　金：佛山市重点专科培育项目资助基金(Fspy3-2015019)

摘　　要：目的:探讨骨假肌源性(上皮样肉瘤样)血管内皮瘤(PHE/ES-HE)的临床病理及影像表现,提高对本病的诊断水平。方法:回顾性分析经病理证实的6例骨原发PHE/ES-HE患者的临床、X线、CT、MRI及病理资料。结果:6例中男4例,女2例,平均年龄31.8岁;主要临床表现为患病部位不同程度疼痛,病程3个月~5年。3例为单发,3例为多发,共31个骨骼病灶。1例病变位于右髂骨,5例位于右侧下肢。所有病灶均表现为溶骨性骨质破坏,边界清楚,增强有明显强化。3个单发病灶的密度、信号不均,边缘有骨质硬化,相邻骨皮质中断,周围形成明显软组织肿块,病灶内部均见残存骨嵴及囊变,1例可见钙化;28个多发病灶中19个密度、信号均匀,15个可见硬化边,内部均无骨嵴、囊变或钙化。6例术前影像学检查均误诊,其中2例误诊为多发性骨髓瘤,2例误诊为骨巨细胞瘤,1例误诊为骨纤维结构不良,1例误诊为上皮样血管内皮瘤。病理标本镜下可见增生的卵圆形或圆形的上皮样细胞及梭形细胞,未见血管腔隙形成,仅1例可见少量胞质内空泡形成,间质内多见散在炎症细胞,免疫组化检查:FLi-1(+),CD31部分(+),FⅧα部分(+),AE1/AE3部分(+),CD34(-)。结论:骨PHE/ESHE的临床病理及影像学表现虽具有一定特征,但术前影像学检查易误诊,需与多发性骨髓瘤、骨巨细胞瘤等疾病鉴别。Objective:To explore the clinical and imaging characteristics of pseudomyogenic(epithelioid sareoma-like)haemangioendothelioma(PHE/ES-HE)of bone.Methods:The clinical data,X-ray,CT and MR images and histopathological appearances of 6 cases with primary skeletal PHE/ES-HE were retrospectively studied,related literature was reviewed,and the imaging and clinicopathological features were summarized.Results:There were 4 males and 2 females with an average age of 31.8 years,pain at the involved area lasted 3 months to 5 years.There were 31 skeletal lesions which involved right ilium and bones of right lower extremity in 6 cases.There were solitary lesions in 3 cases and multifocal lesions in other 3 cases.All of the lesions showed a well-defined osteolysis and marked enhancement after injecting contrast agents.Three solitary lesions demonstrated heterogeneous CT density and MRI intensity with sclerotic rim,cortical defects and large soft tissue mass.Residual bone trabecula and cystic degeneration were present in these 3 lesions,and calcification in one lesion.Among the 28 multifocal lesions,19 lesions showed homogeneous CT density and MRI intensity,15 lesions showed sclerotic rim,and no lesion contained residual bone,cystic degeneration or calcification.There was misdiagnosis of imaging examination in all cases.Among them,2 cases were misdiagnosed as multiple myeloma,2 cases were misdiagnosed as giant cell tumor of bone,one case was misdiagnosed as fibrous dysplasia of bone,and the other one was misdiagnosed as epithelioid hemangioendothelioma of bone.The proliferation of oval cells or circular epithelial-like cells and fusiform cells were seen under the microscope,no vascular lacuna was found.In only one case,the formation of vacuoles was found in the cytoplasm.Some inflammatory cells were scattered in the interstitial tissue.Immunohistochemical staining showed FLi-1(+),CD31(+)partly,FⅧα(+)partly,AE1/AE3(+)partly,Ki-67(+)<20%and CD34(-).Conclusion:Although there are some specific imaging and clinicopathological feat

关 键 词：骨 血管内皮瘤 假肌源性 体层摄影术 X线计算机 磁共振成像 

分 类 号：R445.2[医药卫生—影像医学与核医学]