原发性骨平滑肌肉瘤临床、影像学及病理表现分析  

Radiological and clinical pathological analysis for primary leiomyosarcoma of bone

作　　者：石晓莹[1] 任翠萍[1] 程敬亮[1] 李莹[1] 李贝贝[1] 范伊哲 任仙[2] 

机构地区：[1]郑州大学第一附属医院磁共振科,河南郑州450052 [2]郑州大学基础医学院

出　　处：《实用放射学杂志》2014年第12期2035-2039,共5页Journal of Practical Radiology

摘　　要：目的：分析原发性骨平滑肌肉瘤（PLB）的临床、影像及病理学表现,提高对其诊断水平。方法收集资料完整且经病理证实的 PLB 6例,回顾性分析其影像及临床病理表现,并结合相关文献进行综合分析。结果6例 PLB 患者病变均单发,位于股骨干1例,股骨远端1例,胫骨近端2例,坐骨、肱骨各1例,均侵犯周围软组织形成肿块,无骨膜反应,可见瘤内矿化。其中5例表现为边界不清的溶骨性骨质破坏,1例表现为膨胀性囊状骨质破坏；3例可见硬化边,2例伴有邻近骨皮质增厚；5例 MR T1 WI 呈等信号,T2 WI 呈不均质稍高信号,增强扫描明显不均匀强化；1例呈双环样改变,中心区域 T1 WI、T2 WI 呈不均匀稍高信号,外围环状区域 T1 WI 呈等信号,T2 WI 呈稍高信号,增强扫描外围环状区域明显不均匀强化；免疫组化 SMA 均强阳性,3例 Desmin、Vimentin阳性,2例 Ki-67阳性,CD68散在细胞阳性,S-100均阴性。结论PLB 临床、影像学表现及常规组织细胞学不具特异性,免疫组化和/或超微结构分析显示平滑肌分化可明确诊断。Objective To analyze the clinical,radiological and pathological features of primary leiomyosarcoma of bone and to im-prove the diagnostic level.Methods Totally 6 cases of PLB proved by pathology were collected in our study.The clinical,radiologi-cal and pathological findings were retrospectively analyzed,and combined with the relevant literature to have a comprehensive analy-sis.Results The lesions of 6 patients were solitary,located in femoral （1 case）,distal femur （1 case）,proximal tibia （2 cases）,is-chium （1 case）and humerus （1 case）.There was no periosteal reaction in all 6 cases but they all had soft tissue mass,sometimes there may have some mineralization within the tumor.Ill-defined osteolytic bone destruction was detected in 5 patients,while expan-sion of cystic bone destruction was found in the other 1 patients.3 cases had hardened edge,2 cases with adjacent bone cortical thickening.The MR imaging in 5 patients showed iso-intensity signal on T1 WI,a slightly inhomogeneously higher signal on T2 WI and a significant heterogeneously enhancement after injected of Gd-DTPA.Another 1 patient showed a double ring sign,the central area onT1 WI and T2 WI expressed heterogeneous slightly higher signal,the peripheral annular zone was iso-intensity on T1 WI and slightly higher on T2 WI,after injected contrast medium,it was inhomogeneous enhancement.In immunohistochemically,all tumors reacted positively with SMA,3 patients with Desmin and Vimentin positive,2 patients with Ki-67 positive,and there had some scat-tered cells with CD68 positive,but the S-100 were all negative.Conclusion The clinical,radiological and conventional histological manifestations of PLB are not specific.Immunohistochemical and/or ultrastructural analysis who displayed smooth muscle differenti-ation can confirm the diagnosis.

关 键 词：骨 平滑肌肉瘤 放射摄影术 计算机体层成像 磁共振成像 病理学 

分 类 号：R738.1[医药卫生—肿瘤] R814.4[医药卫生—临床医学]