首页|14例原发性肺淋巴上皮癌的临床病理分析

14例原发性肺淋巴上皮癌的临床病理分析

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背景与目的 原发性肺淋巴上皮癌(primary pulmonary lymphoepithelial carcinoma,PPLEC)是一种罕见的肺部恶性肿瘤,其发病率仅占所有肺恶性肿瘤的0.7%.目前,PPLEC被归类为鳞状细胞癌的特殊亚群.本研究旨在探讨PPLEC的两种亚型在临床病理学上的特征,以提升对该疾病的认识和诊断能力.方法 回顾性分析2019年2月至2023年6月苏州大学附属第一医院经病理学证实为PPLEC的14例患者的临床、病理学、影像学及预后资料,并对相关文献进行复习.结果 PPLEC总计14例,其中Regaud型5例,年龄33-73岁,男性2例,女性3例;Schmincke型9例,年龄36-79岁,男性4例,女性5例.计算机断层扫描(computed tomography,CT)均表现为软组织肿块影或结节影,Reagud型以周围型肿块为主,Schmincke型以中央型为主.病理学示肿瘤细胞呈合胞体样生长,伴随淋巴细胞浸润及间质纤维化,Regaud型边界清晰合并肉芽肿性炎性病变,Schmincke型肿瘤边界模糊.免疫组织化学示肿瘤细胞CK、CK5/6、P40、P63阳性,Regaud型Ki-67增殖指数低于Schmincke型;其中8例接受程序性细胞死亡配体1(programmed death-ligand 1,PD-L1)检测均呈阳性;EB病毒编码的小RNA(Epstein-Barr virus-encoded RNA,EBER)原位杂交检测均阳性;在所有病例中,6例接受手术治疗,8例实施综合治疗,截至随访结束14例患者均存活.结论 PPLEC是一种罕见的肺恶性肿瘤,与EB病毒(Epstein-Barr virus,EBV)感染相关.该疾病可分为Regaud型和Schmincke型,二者在影像学和病理学特征上具有独特差异.在治疗方面,早期以手术治疗为主,中晚期则采取综合治疗,整体预后较好.对于PD-L1高表达的中晚期患者,免疫治疗是一种较为有效的治疗选择.
Clinicopathological Analysis of 14 Cases of Primary Pulmonary Lymphoepithelial Carcinoma
Background and objective Primary pulmonary lymphoepithelial carcinoma(PPLEC)is a rare form of lung malignancy,accounting for only 0.7%of all lung cancers.It is currently classified as a distinct subtype within squamous cell carcinomas.This study aims to explore the clinicopathological characteristics of PPLEC and its subtypes,with the objective of enhancing understanding and improving diagnostic accuracy for this disease.Methods A retrospec-tive analysis was conducted on the clinical,pathological,imaging,and prognostic data of 14 patients diagnosed with PPLEC at the First Affiliated Hospital of Soochow University between February 2019 and June 2023.Results A total of 14 cases of PPLEC were identified,including 5 cases of the Regaud type,with ages ranging from 33 to 73 years,comprising 2 males and 3 females;and 9 cases of the Schmincke type,with ages ranging from 36 to 79 years,including 4 males and 5 females.Computed tomography(CT)scans consistently demonstrated soft tissue masses or nodular shadows.Reagud type mainly showed peripheral masses and Schmincke type mainly showed central masses.Pathological examination revealed tumor cells exhibiting syncytial-like growth,accompanied by lymphocytic infiltration and stromal fibrosis,with the Regaud type showing well-defined borders combined with granulomatous inflammation,while the Schmincke type exhibited indistinct tumor margins.Immunohistochemistry showed that CK,CK5/6,P40 and P63 were positive,and the Ki-67 index of Regaud type was lower than that of Schmincke type;notably,all 8 cases tested for programmed death-ligand 1(PD-L1)were posi-tive.Epstein-Barr virus-encoded RNA(EBER)in situ hybridization was positive in all instances.Among these cases,6 under-went surgical treatment,and 8 received comprehensive therapy;by the end of the follow-up period,all 14 patients remained alive.Conclusion PPLEC is a rare form of malignant lung tumor associated with Epstein-Barr virus(EBV)infection.The Regaud and Schmincke subtypes display distinct imaging and pathological characteristics.In the early stages of the disease,surgical intervention is the primary treatment method;however,for advanced stages,a multimodal treatment approach is utilized,resulting in a relatively favorable prognosis.Immunotherapy represents a promising and effective treatment modal-ity for patients with middle to advanced stage disease exhibiting high PD-L1 expression levels.

Primary pulmonary lymphoepithelial carcinomaSubtypePrognosis

方译萱、王安哲、沈璐敏、袁潇、孔煜

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215006 苏州,苏州大学附属第一医院病理科

原发性肺淋巴上皮癌 亚型 预后

2024

中国肺癌杂志
中国抗癌协会 中国防痨协会 天津医科大学总医院

中国肺癌杂志

CSTPCD北大核心
影响因子:1.397
ISSN:1009-3419
年,卷(期):2024.27(11)