原发性肾滑膜肉瘤及术后复发、转移的影像学表现

目的探讨原发性肾滑膜肉瘤(primary renal synovial sarcoma,PRSS)及术后复发、转移的影像特征,以提高对该病的诊断和鉴别诊断的水平。资料与方法回顾性分析1例PRSS的临床、影像、病理、免疫组织化学以及术后随访资料,结合文献探讨PRSS及其术后复发、转移的影像学征象与临床和病理表现间的关系。结果 CT像上呈不均匀强化的囊实性肿物,大小约10.7 cm×12.3 cm×15.5 cm,肿块外缘可见出血;腰大肌受累;腹膜后未见淋巴结,双肺未发现转移病灶。超声探及右肾回声不均包块,边界略清,内中强回声和多发囊性回声区,多分隔,肿瘤内"星点"状血流信号。镜下瘤细胞呈"梭"形、束状排列,细胞异型,可见核分裂像;免疫组织化学染色:波形蛋白(Vimentin)、CD99、癌基因(Bcl-2)阳性,细胞角蛋白(CK)阴性。术后随访出现局部复发及肺转移,术8个月后死亡。结论 PRSS是一种罕见的恶性肿瘤,预后差,其影像学表现具有一定特征性,但确诊需依靠病理、免疫组织化学和SYT-SSX融合基因检测。CT在诊断肿瘤良恶性、肿瘤分期和随访方面有重要价值。

Primary Renal Synovial Sarcoma With Recurrence and Metastasis

Objective To discuss the imaging features of primary renal synovial sarcoma(PRSS)with recurrence and tastasis and improve its diagnostic accuracy.Materials and Methods The clinical,imaging and pathology findings,immunohistochemistry(IHC) of 1 patient with PRSS were retrospectively analyzed,and the literature was reviewed to evaluate the relationships between the imaging signs,clinical and pathologic findings.Results CT scan:A mass lesion with indistinguishable borders to the kidney,rooting from the right kidney and extending to the inferior and medial aspect anterior to the psoas muscle,about 10.7 cm×12.3 cm×15.5 cm,demonstrating a lobular contour and including cystic areas was detected on CT.No additional tumor,lung metastases,paracaval or paraaortic lymph nodes were detected on CT.A hypoechoic solid mass lesion extending from the lower pole of the right kidney to the inferior and medial aspect,about 13.5 cm×11.1 cm×10.5 cm,demonstrating a lobular contour including cystic areas and star point like flow signals was seen on the ultrasonic examination.The diagnosis of PRSS in the patient was confirmed by postoperative pathology.Under microscope,tumor was typically mitotically active,monomorphic spindle cells growing in intersecting fascicles or in solid sheets with epithelial differentiation.In some areas a haemangiopericytoma like pattern was found.IHC showed that the tumor cells were positive for the markers Vimentin,CD 99 and Bcl 2,but CK was negative.The patient died of local recurrence and multi-metastasis in 8 months after surgery.Conclusion PRSS is extremely rare and malignant tumor with poor prognosis.There are some relatively specific imaging findings,however its diagnosis is depend on pathology,IHC and SYT-SSX gene detection.It has been demonstrated that the use of CT in the diagnosis and the evaluation of the staging of the PRSS is useful by defining lymph node involvement,distant metastases,and local recurrences.