周 旋,王琴琴,刘国伟

背景：原发性肺滑膜肉瘤（PPSS）是一种极为罕见的恶性肿瘤，发病率不足0.5%。其非特异性的临床表现和影像学特征给诊断带来了极大的挑战，患者预后通常较差。病例概述:本文报告了一例61岁男性PPSS患者，主诉胸痛和呼吸困难。影像学检查（CT）显示左下肺存在12×10 cm肿块，确诊过程依赖于组织学和免疫组化分析，组织学表现出典型的滑膜肉瘤特征（如梭形细胞束状排列），免疫组化检测结果显示肿瘤标记物BCL-2、Vimentin、CD99阳性。最终确诊为PPSS。患者接受了三周期EP方案化疗（表柔比星联合顺铂），肿瘤显著缩小后行胸腔镜辅助下左下肺叶切除及纵膈淋巴结清扫术，术后病理切缘显示阴性，显示肿瘤完整切除。讨论：通过PPSS的临床表现、影像学表现、病理学表现、细胞遗传学特点、预后与分级、治疗的探讨，提示临床上PPSS的诊断与治疗需要精准的病理、分子分析以及多学科协作的个体化治疗策略。

原发性肺滑膜肉瘤（PPSS）；诊断；免疫组化；分子病理；综合治疗

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