【摘要】 目的探讨原发性肺滑膜肉瘤(primary pulmonary synovial sarcoma,PPSS)及胸膜转移的影像特征,以提高对该病的诊断和鉴别诊断能力。资料与方法回顾性分析1例PPSS的临床、影像及病理资料,并结合文献探讨PPSS的影像学征象与临床和病理表现间的关系。结果肿瘤为单一肿块,位于中叶边缘,以广基紧贴前胸壁,平扫表现为均质软组织密度,未见钙化和脂肪成分,增强扫描肿块呈中度强化,包膜呈中度环状强化;未见肺门和纵隔淋巴结肿大;发射体层成像(ECT)全身骨扫描未见明显异常;手术病理结果显示肿块有假包膜,灶内见局灶性出血和裂隙状坏死,镜下肿瘤由梭形细胞构成,部分呈血管外皮瘤样结构,细胞异型明显,核分裂易见,免疫组织化学结果显示CD99和波形蛋白(Vim)强阳性,广谱细胞角蛋白(PCK)和上皮膜抗原(EMA)均为少数散在阳性;术后5个月患者因胸痛复查CT发现同侧肋膈角区前、侧、后方多发结节与肿块,转移灶实性部分及包膜均见延时渐进性强化,坏死液化区未见强化。结论 PPSS是一种罕见的恶性肿瘤,预后差,其影像表现具有相对特征性,确诊需依靠病理、免疫组织化学和SYT-SSX融合基因检测。更多还原

【Abstract】 Objective To discuss the imaging features of primary pulmonary synovial sarcoma(PPSS)with multiple pleural metastases and improve its diagnostic accuracy.Materials and Methods The clinical,imaging and pathology findings of 1 patient with primary pulmonary synovial sarcoma were retrospectively analyzed,and the literature was reviewed to evaluate the relationships between the imaging signs,clinical and pathologic findings.Results The tumor was a single mass and had a well-defined pleural-based with homogeneous density in the edge of the middle lobe,with no calcification and a fat component on plain CT scan.On the enhanced CT scan,the tumor showed modately enhancement and circular enhancement with no enlargement of mediastinal and hilar lymphnodes.Skeleton single photon emission CT(ECT) was normal.Gross and histological findings by postoperative pathology showed the tumor had psuedocapsule and was well-circumscribed with foci of necrosis,hemorrhage,and cystic change,tumor was typically mitotically active,spindle cells growing in intersecting fascicles or in solid sheets with epithelial differentiation under microscope.The immunohistochemical examination of CD99,Vim,PCK and EMA were positive.The tumor recurred with multiple pleural nodules and mass at 5 months after surgery,chest CT revealed the tumor was in the ipsilateral costophrenic angle for chest pain of the patient.After dynamic contrast-enhanced,the solid parts and the capsule of the lesion showed progressive enhancement and the liquefied area of the lesion without enhancement.Conclusion Primary pulmonary synovial sarcoma is extremely rare and malignant tumor with poor prognosis.There are some relatively specific imaging findings,however its diagnosis is depend on pathology,immunohistochemistry and SYT-SSX gene detection.更多还原