原发性骨上皮样血管肉瘤的影像学分析

Imaging analysis of primary epithelioid angiosarcoma of the bone

目的探讨原发性骨上皮样血管肉瘤的影像特点，以提高其影像诊断水平。方法回顾性分析6例经病理证实原发性骨上皮样血管肉瘤的影像学表现。其中男2例，女4例，中位年龄51．7岁，病程1～12个月。结果6例原发性骨上皮样血管肉瘤患者均行影像学检查，其中单独行X线平片检查1例，X线平片＋CT1例，X线平片＋MR2例，X线平片＋CT＋MR2例。X线平片及CT表现：5例为单发病灶，1例为多中心发病；5例呈斑片状、地图样的溶骨性骨质破坏，呈轻度膨胀，无硬化边、钙化及骨膜反应；1例呈皂泡状明显膨胀生长，可见基本完整的硬化边，瘤内可见不规则钙化影。MR表现：肿瘤呈等T1、长他信号影，增强扫描肿瘤呈明显不均匀强化，呈等、稍高或低T1WI信号，T2WI高信号，1例瘤内可见小片状长T1、他区。结论原发性骨上皮样血管肉瘤是一种以肿瘤性内皮细胞呈上皮样形态为其特征的高度恶性血管源性肿瘤，影像表现缺乏特征性，确诊依靠病理检查。

Objective To study the imaging features of primary bone epithelioid angiosarcoma, to improve the diagnostic accuracy. Methods Retrospective analysis of 6 cases confirmed by pathology image of primary bone cpithelioid anglosarcoma manifestations. There were 2 male, 4 female, median age 51.7 years, the course of 1--12 months. Results 6 cases of primary bone epithelioid angiosarcoma patients underwent imaging examination, including 1 cases only underwent X-ray ex- amination, X-ray plain film ＋ CT of 1 cases, 2 cases of X-ray plain film ＋ MR, 2 cases of X-ray plain film ＋ CT ＋ MR. The X-ray and CT manifestations ： 5 cases were with single lesion, I cases of multi center for disease ; 5 cases showed patchy, map like osteolytic bone destruction, a mild swelling, no sclerosis, calcification and periosteal reaction; 1 cases were soap bubble obvious expansion growth, hardening visible the basic integrity of the edge, tumor shows irregular calcification. MR features ： the tumor was iso-T1, long T2 signal intensity, enhancement of tumor showed inhomogeneous enhancement, with slightly high or tow signal in T1WI, high signal on T2WI, 1 cases of tumor can be seen in the small pieces of long T1, T2 area. Conclusion Primary bone epithelioid angiosarcoma is a kind of tumor that endothelial cells were epithelioid morphology as characterized and highly malignant vascular tumors, imaging showed lack of characteristic, diagnosis depends on pathological examina- tion.