原发性气管淋巴上皮瘤样癌临床分析

The clinical analysis of primary lymphoepithelioma-like carcinoma of the trachea

目的探讨原发性气管淋巴上皮瘤样癌的临床特征、诊断、治疗及预后。方法回顾性分析一例原发性气管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。结果胸部CT提示气管狭窄,电子支气管镜检査提示气管中段环状狭窄,病理见癌细胞呈巢状聚集成堆,肿瘤间质有淋巴细胞浸润,免疫组化EB病毒编码小RNA(EBER)阳性,排除鼻咽癌后确诊为原发性气管淋巴上皮瘤样癌。因患者无手术机会给予气管Y型硅酮支架置入,患者拒绝放化疗,目前随访11个月病情稳定。文献复习提示原发性气管淋巴上皮瘤样癌多见于年轻患者,无性別差异,其发生与EB病毒感染密切相关,临床表现缺乏特异性,多以呼吸困难为首发症状,病理及免疫组化是其主要确诊方法。该疾病宜采用手术为主的综合治疗。结论原发性气管淋巴上皮瘤样癌是一种罕见、独特类型的气管肿瘤,其发生与EB病毒感染密切相关,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 analyze the clinical characteristics,diagnosis,treatment and prognosis of lymphoepithelioma-like carcinoma of the trachea.Methods A case of lymphoepithelioma-like carcinoma of the trachea was reported and related literature was analyzed and summarized.Results Chest CT and electronic bronchoscopy revealed tracheal stenosis.Histologically,the tumor cells showed nodular growth with surrounding lymphocytic infiltration in the stroma and immunohistochemical evidence of EBER(Epstein-Barr virus-encoded RNA)was positive.The patient was finally diagnosed with lymphoepithelioma-like carcinoma of the trachea excluding the diagnosis of nasopharyngeal cancer.A Y-shaped silicone stent was implanted because of limited resection.He refused further treatment such as chemotherap,radiotherapy and remained stable with 11-month follow-up.Literature review showed lymphoepithelioma-like carcinoma of the trachea was more common in the younger people without a sex prediction,where there was a definite association with the EBV infection.The clinical findings were nonspecific commonly with dyspnea as initial manifestation.The diagnosis was confirmed with pathologic and immunohistochemical evidence.The combined therapy mainly including surgery should be adopted in the patients.Conclusion Lymphoepithelioma-like carcinoma of the trachea is a rare and unique type of tracheal tumor.The tumor has a strong association with the EB virus.The combined therapy mainly including surgery should be adopted in the patients.Prognosis of patients undergoing radical surgery is well.