肺纤毛黏液结节性乳头状肿瘤的临床病理特征

Clinicopathologic characteristics of lung ciliated muconodular papillary tumor

目的:探讨肺纤毛黏液结节性乳头状肿瘤(ciliated muconodular papillary tumor,CMPT)的临床病理特征。方法:选取2017年1月至2018年12月大连大学附属新华医院及大连医科大学附属第一医院收治的8例临床资料完整的CMPT,分析其临床特征、病理组织学特点、免疫表型、分子病理及鉴别诊断。结果:8例患者中,男女比例为1∶7,平均年龄63.5岁,无优势性别及发病部位。首发症状均为体检发现肺占位,影像学表现为磨玻璃影(GGO)。组织学上肿瘤由纤毛细胞、基底细胞及黏液细胞组成,可见黏液湖形成及乳头状结构。免疫组化显示纤毛柱状细胞和黏液细胞细胞角蛋白(CK7)(+),甲状腺转录因子(TTF-1)(+),NapsinA(+或-);基底细胞P63(+)、P40(+)、CK5/6(+);肿瘤细胞多数弱表达表皮生长因子受体(EGFR)(+),6例中有1例EGFR 19号外显子缺失(1/6,17%)。黏液染色阿利新蓝-过碘酸雪夫(AB-PAS)(+),弹力纤维染色显示肺泡弹性框架结构缺失或破坏。治疗方法均为手术切除,术后无复发转移。结论:CMPT是一种罕见的良性肿瘤,易误诊为黏液腺癌,通过正确识别纤毛细胞和基底细胞的连续性表达,可降低该病的误诊率。

Objective:To explore the clinicopathologic features of ciliated muconodular papillary tumors(CMPT).Methods:Eight CMPT patients with complete clinical data were selected from Xinhua Hospital,Dalian University and the First Affiliated Hospital of Dalian Medical University from January 2017 to December 2018,the clinical characteristics,histopathological features,immunophenotype,molecular pathology,and differential diagnosis were analyzed.Results:Among the 8 patients,the ratio of male to female was 1∶7,and the mean age was 63.5 years,there was no dominant sex or location.The first symptoms were all lung space occupying found in physical examination,and imaging manifestations were ground-glass opacity(GGO).Histologically,the neoplasm is composed of ciliary cells,basal cells,and mucous cells.Note the lacustrine formation and papillary structures.Immunohistochemistry showed ciliary columnar cells and mucous cells cytokeratin(CK7)(+),thyroid transcription factor-1(TTF-1)(+),NapsinA(+or-);Basal cells P63(+),P40(+),CK5/6(+);epidermal growth factor receptor(EGFR)(+)was weakly expressed in most of the tumor cells,and EGFR was detected by AMRS PCR.Exon 19 was absent in 1 of 6 cases(1/6,1.7%).AB-PAS(+)with mucous staining,and elastic fiber staining showed loss or destruction of the alveolar elastic framework.All cases were treated by surgical resection without recurrence or metastasis.Conclusions:CMPT is a rare benign tumor,which is easy to be misdiagnosed as mucinous adenocarcinoma.By correctly recognizing ciliated cells and the continuous expression of basal cell markers,the misdiagnosis rate of the disease can be reduced.