目的:探讨硬化性肺细胞瘤 (pulmonary sclerosing pneumocytoma, PSP) 的临床病理特征、免疫表型、诊断及鉴别诊断.方法:对8例PSP的临床资料、组织学特征及免疫表型进行分析, 并结合文献进行总结.结果:肿瘤7例位于右肺, 1例位于左肺, 术前CT无一例明确诊断.镜检:肿瘤均由两种细胞、四种结构构成, 两种细胞即表面细胞及间质细胞, 四种结构为实性区、乳头状区、血管瘤样区及硬化区.免疫表型:表面细胞及间质细胞均表达EMA和TTF1;表面细胞表达AE1/AE3、CK7、Napsin A;间质细胞表达Vimentin.结论:PSP缺乏特征性的临床和影像学表现, 术前不易明确诊断, 而镜下"四种结构、两种细胞"及特征性的免疫表型是其明确诊断的重要依据.%Objective: To investigate the clinicopathologic features, immuophenotype, diagnosis and differential diagnosis of pulmonary selerosing pneumocytoma (PSP) . Methods: Data of 8 cases of PSP were analyzed including clinical data, pathological feature and immuophenotype and the related literature was reviewed. Results: 7 cases of PSP occurred in the right lung and 1 case in the left lung. But none of patient was diagnosed definitively by CT before operation. Under light microscope the tumors show two kinds of tumor cells and four typical patterns. Two kinds of cells were surface cells and round cells. Four typical patterns were solid, papillary, hemorrhagic and sclerotic area. On immuophenotype, both surface cells and round cells were EMA and TTF1 positive. Surface cells expressed AE1/AE3, CK7 and Napsin A. Round cells expressed Vimentin. Conclusion: PSP lacks special clinical and imaging manifestation and has a high misdiognosis rate before operation. Two kinds of cells and four typical paterns under light microscope and it's typical immuophenotype are the key of PSP's diagnosis.
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