原发性肺淋巴上皮瘤样癌一例并文献复习  

Primary lymphoepithelioma-like carcinoma of the lung:a case report and literature review

作　　者：宋琳[1] 韩锋锋[1] 崔志磊[1] 管雯斌[2] 郭雪君[1] 

机构地区：[1]上海交通大学医学院附属新华医院呼吸科,上海200092 [2]上海交通大学医学院附属新华医院病理科,上海200092

出　　处：《中国呼吸与危重监护杂志》2017年第6期575-580,共6页Chinese Journal of Respiratory and Critical Care Medicine

基　　金：国家自然科学基金(81570030)

摘　　要：目的 探讨原发性肺淋巴上皮瘤样癌的临床病理特征、诊断、治疗及预后。方法 回顾性分析1例原发性肺淋巴上皮瘤样癌患者的临床资料并结合相关文献进行总结。结果 患者,男,36岁,因发热伴咳嗽、咳痰、活动后气促1个月余就诊。胸部CT提示左上肺占位伴左肺不张。电子支气管镜检查可见左主支气管内新生物,病理见癌细胞呈合胞体状聚集成堆,细胞核呈空泡状,肿瘤间质有淋巴细胞浸润,免疫组化EB病毒(EpsteinBarr virus,EBV)编码小核RNA阳性,排除鼻咽癌后确诊为原发性肺淋巴上皮瘤样癌。给予患者泰素帝联合奈达铂方案化疗6次,化疗疗效评估病情稳定。但化疗结束后1个月患者再次出现气促症状,胸部CT检查提示病灶进展伴有大量心包积液、胸腔积液,患者无法耐受化疗,仅接受对症支持治疗,最终死亡。检索到符合要求的中文文献18篇、外文文献25篇。文献复习提示原发性肺淋巴上皮瘤样癌多见于不吸烟的年轻患者,无性别差异,其发生与EBV感染密切相关,临床表现缺乏特异性,病理组织学及免疫组化是其主要确诊方法。该疾病对放化疗较敏感,宜采用手术为主的综合治疗。早期及可手术切除的患者预后较好。无法行手术切除的患者预后较差。结论 原发性肺淋巴上皮瘤样癌的发生与EBV感染密切相关,病理组织学及免疫组化是主要的确诊方法该疾病对放化疗较敏感,宜采用手术为主的多学科综合治疗。早期及可手术切除的患者预后较好。Objective To investigate the clinicopathologic features, diagnosis, treatment and prognosis of the primary lymphoepithelioma-like carcinoma （PLELC） of the lung. Methods Clinical and pathological data of one patient with PLELC of the lung were reviewed. PLELC of the lung was analyzed through review of literatures. Results A mass in the upper lobe of the left lung and atelectasis were detected by CT in a 36 year old nonsmoker male. He presented with cough, expectoration, fever and shortness of breath for one month. Neoplasm in left main bronchus was observed through bronchoscopy. Histological study showed cohesive clusters of comprising syncytial-appearing cells with vesicular nuclei and tumor infiltrating lymphocytes. Immunohistochemistry showed positive expression of Epstein-Barr virus （EBV） encoded small nuclear RNA. Nasal CT scan was negative for suspicious nasopharyngeal mucosal lesion or tumor. Then he was diagnosed as PLELC. The patient received docetaxel＋nedaplatin regimens for 6 cycles with stable disease, but one month later after the end of chemotherapy, he was readmitted to hospital for dyspnea. CT scans showed progression of the lesion with massive pericardial effusion and pleural effusion. The patient could not tolerate chemotherapy, then received supportive treatment and died soon. Totally 18 Chinese articles and 25 foreign articles were screened out. Literature review suggested that patients with PLELC of the lung usually occurred in young nonsmokers and without gender difference. PLELC of the lung was closely associated with EBV infection. Its clinical manifestations were not specific, so that histopathological and immunohistochemical analyses were the main method of diagnosis. PLELC of the lung may respond to chemotherapy and radiotherapy. Surgery-based multimodality treatment was recommended. Patients with early or resectable disease had better prognosis, but patients with unresectable disease had poor prognosis. Conclusions PLELC of the lung may be closely associated with EBV infection

关 键 词：肺淋巴上皮瘤样癌 临床病理学 EB病毒 预后 

分 类 号：R734.2[医药卫生—肿瘤]