肺原发性腺样囊性癌的临床病理特征分析  

Clinicopathologic Study of Pulmonary Primary Adenoid Cystic Carcinoma

作　　者：杨朝晖[1,2] 王建军 何燕 余波 陆珍凤 徐艳 周晓军 石群立 

机构地区：[1]南京军区南京总医院病理科南京大学医学院临床学院,江苏省南京市210002 [2]浙江省台州市中心医院病理科

出　　处：《中国全科医学》2015年第24期2961-2965,共5页Chinese General Practice

基　　金：国家自然科学基金资助项目(81171391)

摘　　要：目的分析肺原发性腺样囊性癌的临床病理特征,并探讨其诊断标准及预后。方法选取2002年1月—2014年1月于南京军区南京总医院行胸部CT或支气管镜检查并诊断为肺原发性腺样囊性癌的患者16例。记录患者的一般资料,包括性别、年龄、临床TNM分期、肿瘤发生部位、临床表现、影像学表现、确诊及治疗方法;病理学检查,包括巨检、镜下特点、免疫表型;检测表皮生长因子受体(EGFR)热点突变基因(19、21外显子)的突变情况。从手术切除及穿刺活检标本确诊后开始电话或门诊随访,半年随访1次,随访至2014-07-31,随访内容为是否放射治疗、是否化学治疗、是否复发转移及死亡日期。结果肿瘤多发生在气管或主支气管,临床表现为咳嗽及痰中带血,偶有胸痛、胸闷、呼吸困难和发热等症状。影像学表现,10例患者胸部CT检查示:气管或主支气管内、肺门及肺叶结节影或类圆形高密度影,边缘欠清晰;1例支气管镜检查示:左主支气管内新生物,肿瘤表面较光滑。巨检显示气管或主支气管内、肺门和被膜下见肿块,大小1.2 cm×1.2 cm×1.0 cm^6.2 cm×2.5 cm×2.0 cm,切面灰白、实性,质地中等,部分有黏液感,边界不清,其中1例侵及邻近胸膜。镜下特点:癌细胞呈腺管样、筛状、小梁状或巢状排列,腺腔或筛孔中可见均质淡蓝色黏液,癌细胞体积小,大小较一致,细胞质少,细胞卵圆形到多角形,核染色深,核分裂象不常见;肿瘤组织可或不累及气管。癌细胞角蛋白(CK)8/18、CK34βE12、平滑肌肌动蛋白(SMA)、p63、ki-67阳性表达。1例患者行EGFR热点突变基因检测示:19、21外显子未发生突变。生存时间为5~96个月,平均44个月。6例局部复发或伴有肺门、纵隔淋巴结及骨转移。结论肺原发性腺样囊性癌是一种少见的低度恶性肿瘤,组织形态学及生物学行为与涎腺的腺样囊性癌相似。确诊主要靠组织病理学检查,并�Objective To investigate the clinical and pathological characteristics of primary pulmonary adenoid cysticcarcinoma and explore its diagnostic criteria and prognosis .Methods 16 patients diagnosed as pulmonary primary adenoid cystic carcinoma by chest CT or bronchoscopy test in the Nanjing General Hospital of Nanjing Military Command were enrolled in the study from January 2002 to January 2014.General information , including gender , age, clinical TNM staging , tumor location, clinical manifestations, imaging manifestations, diagnostic and therapeutic methods , were documented.Pathological features were inspected by gross investigation , microscopy and immunophenotype analysis .The mutations of hot -spot-mutation exons （19th, 21th exon） of epidermal growth factor receptor （EGFR） gene were determined.The telephone or outpatient follow-ups were carried out from confirmed surgical diagnosis to 31 July 2014, with a period of half year.Items of follow-up included whether the patients received radiotherapy or chemotherapy , whether tumors metastasized and the date of death .Results Tumors tended to occur in trachea and primary bronchus .Cough and bloody sputum were main symptoms , while chest pain , chest＆amp;nbsp;distress, breathing difficulty and fever were occasionally seen .Chest CT scanning demonstrated that 10 of the 16 patients had shadows or quasi -circular high density shadows in rachea , primary bronchus , hiluspulmonis and lung nodules , with fade border.Bronchoscopy showed that a neoplasm sprout out of left primary bronchus in one patient but the surface was smooth .Gross investigation showed there were lumps in rachea , primary bronchus , hiluspulmonis and under envelopes , with the size of 1.2 cm ×1.2 cm ×1.0 cm-6.2 cm ×2.5 cm ×2.0 cm.The cross sections of lumps were white , solid, moderate -textured, partly sticky and with blurring borders .In one case , the lumps have infiltrated into adjacent pleura .Microscopy showed that cancer cells distributed as adenoid tubular like , cribrose , trab

关 键 词：肺肿瘤 癌  腺样囊性 病理学  临床 预后 

分 类 号：R734.2[医药卫生—肿瘤]