肺纤毛黏液结节性乳头状肿瘤4例临床病理观察  

Clinicopathological features of ciliated muconodular papillary tumor of the lung:report of four cases

作　　者：李佳 相龙全 武迎 关春艳 董格红 LI Jia;XIANG Longquan;WU Ying;GUAN Chunyan;DONG Gehong(Department of Pathology,Beijing Tiantan Hospital Affiliate to Capital Medical University,Beijing 100070,China;Department of Pathology,the First People's Hospital of Jining,Jining,Shandong 272000,China)

机构地区：[1]首都医科大学附属北京天坛医院病理科,北京100070 [2]济宁市第一人民医院病理科,山东济宁272000

出　　处：《安徽医药》2024年第11期2278-2281,I0009,共5页Anhui Medical and Pharmaceutical Journal

摘　　要：目的深入分析肺纤毛黏液结节性乳头状肿瘤(CMPT)的临床表现、病理学征象、免疫组化特征,并讨论其与其他病变的鉴别诊断。方法回顾性分析2018年7月至2022年12月首都医科大学附属北京天坛医院确诊的4例肺CMPT病人的临床病理资料,分析其临床特点、影像学特征、组织学特点及免疫表型,并结合相关文献进行探讨。结果病人年龄范围为55~67岁,男性2例,女性2例,均无呼吸道临床症状,无吸烟史。2例发生于左肺,2例发生于右肺,均接受了楔形肺切除术或肺段切除术。大体检查肿瘤长径范围为4~7 mm。显微镜下观察,肿瘤呈腺样或乳头状的结构,细胞类型包括纤毛柱状细胞、黏液细胞以及基底细胞。肿瘤内部及其周围的肺泡腔内充满了黏液。细胞形态保持一致,未观察到细胞异型性、核分裂象或坏死。免疫组化检测结果显示,纤毛柱状细胞和黏液细胞均呈细胞角蛋白7(CK7)阳性表达,部分细胞表达甲状腺转录因子-1(TTF-1),而天冬氨酸蛋白酶A(Napsin A)表达阴性或弱阳性。基底细胞则表达P63、P40和CK5/6。Ki-67的增殖指数相对较低,不超过5%。基因检测结果显示2例样本中检出EGFR基因突变。结论肺CMPT是罕见的肺原发良性肿瘤,其组织形态及免疫表型均具有特征性,但在影像学检查及术中冰冻病理检查中极易误诊为腺癌,需要在石蜡切片中仔细寻找CMPT的特点并结合免疫组化特征进行诊断,该肿瘤预后良好。Objective To investigate the clinical manifestations,pathological signs and immunohistochemical characteristics,and to discuss differential diagnosis between ciliated muconodular papillary tumor(CMPT)and other lesions.Methods Four cases of CMPT were retrospectively analyzed for clinicopathologic features,radiographic characteristics,histologic features,and immunophenotype in Beijing Tiantan Hospital Affiliate to Capital Medical University from July 2018 to December 2022,and were discussed in conjunction with the literatures.Results The patient's age ranged from 55-67 years,with 2 males and 2 females.Neither of them had respiratory symptoms or smoking history.2 cases occurred in the left lung and 2 in the right lung,all of them performed wedge pneumonectomy or segmentectomy.Grossly,the maximum diameters of the tumor ranged between 4 to 7 mm.Microscopically,the tumors arrayed tubular and papillary structures,and were comprised of mucous cells,ciliated cells and basal cells.Mucus was abundant both inside and in the surrounding alveolar cavities of the tumor.Cell morphology remained consistent and lacked atypia,and no obvious mitosis/necrosis were detected in the tumor.Immunohistochemistry analysis showed strong positive for CK7,partial positive for TTF-1 while negative for Napsin A.The basal cells expressed P63,P40 and CK5/6.The Ki-67 proliferation index was less than 5%.Genetic testing revealed that EGFR mutations were detected in two of these samples.Conclusions The CMPT of the lung is a rare benign tumor with typical pathological and immunochemistry features.However,it is easily distinguished as adenocarcinoma in imaging examination and intraoperative frozen pathological examination.The characteristics of CMPT should be carefully researhed in paraffin sections and combined with immunohistochemical characteristics for diagnosis.The tumor has a good prognosis.

关 键 词：肺肿瘤 纤毛黏液结节性乳头状肿瘤 细支气管腺瘤 基底细胞 纤毛细胞 

分 类 号：R734.2[医药卫生—肿瘤]