原发性肺淋巴上皮瘤样癌18例临床特征分析

Clinical analysis of primary pulmonary lymphoepithelioma-like carcinoma in 18 patients

目的分析原发性肺淋巴上皮瘤样癌(PPLELC)的临床特点、治疗及预后。方法回顾性分析2014年1月至2021年7月在南京医科大学第一附属医院经病理确诊的18例PPLELC患者的临床资料, 其中男10例, 女8例, 年龄48~74(56.4±6.3)岁, 对其临床症状、实验室检查结果、影像学特征、治疗和转归进行综合分析。结果 18例患者中2例有吸烟史。多数患者无明显临床症状, 部分表现为咳嗽(4/18)、胸闷(2/18)或胸痛(1/18)。血肿瘤标志物CEA多正常, 部分患者NSE(7/16)或CYFRA21-1(4/16)升高。18例患者TNM分期以Ⅰ期(12/18)为主, 病灶皆为单发, 多位于右肺(10/18), 周围型(13/16)常见, 可伴有分叶(9/16)或空洞(2/16)。6例患者行PET/CT检查, SUV最大值为(8.15±3.08), MTV值为(6.85±4.95)cm^(3)。10例患者行驱动基因检测, 其中6例行间变性淋巴瘤激酶(ALK)检测, 仅1例(1/6)ALK突变阳性。手术切除或手术联合化疗、免疫治疗是其主要治疗方式。至随访截止时间, 18例患者平均生存时间(26.0±12.7)个月, 仅1例死亡(生存时间为24个月), 研究队列未达到中位总生存期(OS), 2年OS率为87.5%。结论 PPLELC以右肺多见, 周围型为主, 驱动基因突变发生率低, PET/CT有助于早期诊断, 多数病例发现时处于早期, 手术切除或手术联合多模式治疗是其主要治疗方式。

Objective To describe the clinical features,treatment and prognosis of primary pulmonary lymphoepithelioma-like carcinoma(PPLELC).Methods We conducted a retrospective analysis of 18 patients diagnosed with PPLELC from January 2014 to July 2021 in the First Affiliated Hospital of Nanjing Medical University.Clinical symptoms,lab data,imaging characteristics,treatment and prognosis were reviewed and analyzed.Results This study consisted of 10 men and 8 women,with a mean age of(56.4±6.3)years,and two of them had a history of smoking.Most of them had no symptoms,while others showed some symptoms including cough(4/18),chest tightness(2/18)or chest pain(1/18).Serum levels of carcinoembryonic antigen(CEA)were normal(17/17),while serum levels of neuron-specific enolase(NSE)(7/16)and CYFRA21-1(4/16)were increased,respectively.According to the eighth edition of international TNM staging standard for lung cancer,12 of 18 patients were classified as stageⅠ.The lesions were solitary,mostly in the right lung(10/18),and peripheral type(13/16)were common,which could be accompanied by lobularization(9/16)or cavitation(2/16).The PET/CT data of 6 patients showed that the maximum standardized uptake value(SUVmax)was(8.15±3.08)and the metabolic tumor volume(MTV)was(6.85±4.95)cm^(3).Ten patients(10/18)received gene tests.Among them,6 cases were detected for ALK,and only one of them was positive for ALK mutation.The pathology of PPLELC was similar to that of undifferentiated nasopharyngeal carcinoma and it should be differentiated from ordinary lung squamous cell carcinoma according to EBER detection.Surgical resection or surgery combined with chemotherapy and immunotherapy were the main treatments in this study.Up to the follow-up time,the average survival time of the 18 patients was(26.0±12.7)months,and only 1 patient died(overall survival time of 24 months),and the median overall survival(OS)was not reached,with a 2-year OS rate of 87.5%.Conclusions PPLELC usually occurred in the right lung and belonged to the peripheral type.The gene mutation was rare.PET/CT is helpful for early diagnosis and most patients can be diagnosed in the early stage.Surgery and surgery combined with multimodality therapies are the main treatments.