肺原发性X淋巴上皮瘤样癌的临床特征、治疗及预后分析。
Analysis of clinical features, treatment, and prognosis of primary Xlymphoepithelioma-like carcinoma of the lung.
作者信息
Hu H-Y, Long L, Dai S-A, Yan G-Q, Huang Y, He J
机构信息
State Key Laboratory of Biotherapy, Lung Cancer Center, West China Hospital of Sichuan University, Chengdu, Sichuan, China.
出版信息
Eur Rev Med Pharmacol Sci. 2022 Dec;26(24):9416-9425. doi: 10.26355/eurrev_202212_30693.
OBJECTIVE
Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is a rare lung malignancy occurring most frequently in young non-smokers from Southeast Asia. Given its low incidence, PPLELC clinical features, treatment methods, and the factors affecting its prognosis remain elusive. To date, PPLELC data are mainly derived from clinical case reports, and no cohort studies are available. Therefore, we retrospectively analyzed a group of PPLELC cases and summarized the clinical features of patients, treatment responses, and the factors affecting patient prognosis.
PATIENTS AND METHODS
A total of 91 patients having primary pulmonary lymphoepithelioma-like carcinoma were recruited in this study. These included sex, age, place of birth, smoking history, pre-treatment symptoms, tumor location, tumor markers, maximum tumor diameter, treatment regimen, lymph node presence metastasis after an operation, pathological picture, immunohistochemistry, genetic findings, and tumor stage grading. We determined the overall survival (OS), progression-free survival (PFS), basic clinical characteristics, treatment option, treatment response, and recurrence pattern among the patients. In addition, we understood the influence of sex, age, tumor, nodes, and metastases (TNM) stage, tumor size, and surgery over patient prognosis.
RESULTS
Primary pulmonary lymphoepithelioma-like carcinoma is more common among young non-smokers, with a slightly higher incidence in women than in men. The expression of Epstein-Barr virus-encoded small RNA (EBER), pancytokeratin (PCK), Cytokeratin 5/6 (CK5/6), and tumor protein 63 (P63) was positive in immunohistochemistry. Serum cytokeratin 19 fragment antigen (CYFRA21-1) and Epstein-Barr DNA (EB-DNA) could be used as markers to diagnose primary pulmonary lymphoepithelioma-like carcinoma. TNM stage and surgery were independent prognostic factors.
CONCLUSIONS
Primary pulmonary lymphoepithelioma-like carcinoma is rare, showing a good prognosis.
目的
原发性肺淋巴上皮瘤样癌（PPLELC）是一种罕见的肺恶性肿瘤，最常见于东南亚年轻非吸烟者。鉴于其发病率低，PPLELC的临床特征、治疗方法及影响其预后的因素仍不明确。迄今为止，PPLELC的数据主要来自临床病例报告，尚无队列研究。因此，我们回顾性分析了一组PPLELC病例，总结了患者的临床特征、治疗反应及影响患者预后的因素。
患者与方法
本研究共纳入91例原发性肺淋巴上皮瘤样癌患者。这些信息包括性别、年龄、出生地、吸烟史、治疗前症状、肿瘤位置、肿瘤标志物、最大肿瘤直径、治疗方案、术后淋巴结转移情况、病理图像、免疫组化、基因检测结果及肿瘤分期分级。我们确定了患者的总生存期（OS）、无进展生存期（PFS）、基本临床特征、治疗选择、治疗反应及复发模式。此外，我们了解了性别、年龄、肿瘤、淋巴结及转移情况（TNM）分期、肿瘤大小及手术对患者预后的影响。
结果
原发性肺淋巴上皮瘤样癌在年轻非吸烟者中更为常见，女性发病率略高于男性。免疫组化中，爱泼斯坦-巴尔病毒编码的小RNA（EBER）、全细胞角蛋白（PCK）、细胞角蛋白5/6（CK5/6）和肿瘤蛋白63（P63）表达呈阳性。血清细胞角蛋白19片段抗原（CYFRA21-1）和爱泼斯坦-巴尔病毒DNA（EB-DNA）可作为诊断原发性肺淋巴上皮瘤样癌的标志物。TNM分期和手术是独立的预后因素。
结论
原发性肺淋巴上皮瘤样癌罕见，预后良好。
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