四肢腺泡状软组织肉瘤影像学表现与病理组织学研究

Imaging Findings and Pathological Features of Alveolar Soft Tissue Sarcoma of Extremities

目的回顾性分析腺泡状软组织肉瘤的影像学征象及病理组织学特点,总结其特征,以提高对本病的认识和诊断水平。方法搜集2000年至2012年经病理组织学证实的腺泡状软组织肉瘤6例,分析其临床资料及影像、病理资料。结果 6例腺泡状软组织肉瘤分别发生于大腿、髂部、前臂、腋部及膝部,其中2例诊断时已分别发生肝脏和肺部转移。X线检查帮助不大,CT上见分叶状或不规则状软组织肿块。MRI上信号不均匀,大部分内部见索条状＂信号流空＂,增强扫描中等~明显强化,3例周围见延迟强化的迂曲血管。结论四肢腺泡状软组织肉瘤无特异性影像学表现,MRI能提供相对较多信息,较大软组织肿块内部见到索条状＂血管流空＂及肿块周围见到延迟强化血管,对诊断有提示作用。

Objective To retrospectively analyze the imaging manifestations and histopathological features of alveolar soft tissue sarcoma in order to improve the understanding of this disease. Methods Six patients with pathologically-proved alveolar soft tissue sarcoma, who were encountered in authors＇ hospital during the period from 2000 to 2012, were enrolled in this study. The clinical data, the imaging findings and pathological results were retrospectively analyzed. Results The lesions were separately located at the thigh, iliac, forearm, axilla and knee. Hepatic or pulmonary metastasis was noted in two patients when the diagnosis was made. X-ray had little help for the diagnosis of the disease. On CT scans the lesion presented as irregular soft tissue mass with lobulated margin. On MRI, the lesion exhibited inhomogeneous signal, with streak-shaped “signal flow void” sign being seen within most lesions. Moderate to strong enhancement of the lesion was dis- played on contrast-enhanced scans. Delayed enhanced tortuous vascular shadow was seen in three cases. Conclusion The alveolar soft tissue sarcoma has no specific imaging manifestations MRI can provide some useful signs to help make the di- agnosis, such as streak-shaped “signal flow voids” sign within the mass, delayed enhanced tortuous vascular shadow a- round the mass, etc.