软组织透明细胞肉瘤的临床、MRI和病理学分析及文献复习

Clinical,MRI and Pathological Analysis of Clear Cell Sarcoma of Soft Tissue and Literature Review

目的分析软组织透明细胞肉瘤(CCSST)的临床、MRI和病理学表现。方法回顾性分析5例经病理证实的CCSST的临床资料,并复习相关文献,观察其临床、MRI及病理学特点。结果 5例CCSST,肿瘤分别位于大腿、足部、肩部、肘部及腰背部。5例均接受MRI平扫及增强扫描,T_1WI序列上,3例肿瘤信号稍高于肌肉信号,2例合并出血呈高低混杂信号;T_2WI序列上,4例肿瘤均表现为高低混杂信号,1例肿瘤表现为均匀高信号;增强扫描4例不均匀强化,内见斑点状无强化出血区,1例明显均匀强化。3例区域淋巴结转移,1例肺转移,1例术后复发。结论 CCSST是一种罕见的恶性软组织肿瘤,好发于中青年四肢,MRI多表现为软组织深部混杂信号软组织肿块,常伴区域淋巴结转移,术后易复发,综合临床及MRI特点,对其诊断具有一定的意义。

Objective To study the clinical,MR imaging and pathological features of clear cell sarcoma of soft tissue（ CCSST）,in order to achieve more information about the disease and improve the diagnosis. Methods A total of five patients with CCSST were collected. The clinical manifestations,MRI and pathologic appearances of the five cases with CCSST were retrospectively analyzed and the related literatures were reviewed. Results In five cases of CCSST,three cases were male and the other two were female. Tumors were located in the thigh,foot,shoulder,elbow and lower back,one case respectively. All the patients underwent plane and contrast-enhanced MR scan. Compared with the muscle signal in MR imaging,three lesions presented a slightly hyperintense signal,the other two lesions with hemorrhage presented mixed intensity signal on T1-weighted images. Four cases showed mixed intensity signal and one case showed homogeneous hyperintensity signal on T2-weighted images. Four cases showed conspicuous inhomogeneous enhancement and the other one showed homogeneous enhancement. Of the five cases,there were three cases with regional lymph node metastasis,one case with lung metastasis and one case was postoperative recurrence. Conclusion CCSST is a rare malignant soft tissue tumor.It affects predominantly young to middle-aged adults and mostly arises in the deep soft tissues of the limbs. The MRI imaging of CCSST is predominantly displays a mixed signal intensity soft tissue tumor with regional lymph nodes metastasis.Combining the clinical and MR imaging can improve the diagnosis of the disease.