36例肺硬化性肺细胞瘤的临床病理及免疫表型

Clinicopathological and immunophenotypic analysis of 36 cases of pulmonary sclerosing pneumocytoma

目的:探讨肺硬化性肺细胞瘤(pulmonary sclerosing pneumocytoma,PSP)的临床病理及免疫表型特征。方法:收集36例符合入组要求的PSP患者的临床病理资料,分析该疾病的临床表现、影像学特点、病理学特点以及免疫表型。结果:PSP多见于中年女性,常无症状,影像学结果大多表现为边界清晰的圆形或类圆形结节。病理关键特征为肿瘤界清、2种肿瘤细胞及4种组织结构。免疫表型:两种细胞均表达TTF-1、EMA、p-AKT;表面细胞表达CK、NapsinA;间质细胞表达vimentin,部分表达ER、PR;均不表达BRAF V600E。术后随访未见复发及转移。结论:PSP的临床及影像学表现缺乏特异性,病理形态复杂多样,掌握关键形态特征及结合免疫组织化学结果有助正确诊断,预后良好。

Objective:To explore the clinicopathological characteristics and immuophenotypes of pulmonary sclerosing pneumocytoma(PSP).Methods:The clinicopathological data of 36 PSP patients meeting the enrollment requirements were collected.The clinical manifestations,imaging and pathological characteristics,and immunophenotypes of the disease were analyzed.Results:PSP was most commonly seen in middle-aged females.Patients were typically asymptomatic.Imaging examination revealed that PSP presented with a solitary wellcircumscribed mass.The key pathological features included circumscribed lesion,two types of tumor cells and four typical histological patterns.By immunohisto chemistry,two types of tumor cells expressed TTF1,EMA and p-AKT.Additionally,cuboidal cells expressed CK,whereas round cells were positive for vimentin and usually positive for ER,PR.Neither of them expressed BRAF V600E.All the patients had no recurrence and metastasis during the follow-up.Conclusion:PSP lacks special clinical and imaging manifestations,but having diverse histological patterns.The pathological diagnosis depends on the key morphologic features including two types of tumor cells and four patterns and immunohistochemical staining.PSP clinically behaves in a benign fashion.