11例原发性肺淋巴上皮瘤样癌的临床病理分析

Clinicopathological analysis of primary pulmonary lymphoepithelioma-like carcinoma in 11 patients

目的探讨原发性肺淋巴上皮瘤样癌(PPLELC)的影像学及临床病理表现,以提高对该疾病的认识和诊断水平。方法回顾性分析2017年1月至2022年12月经手术病理证实的11例PPLELC患者的临床表现、影像学和组织病理学特征以及治疗和预后情况。结果11例患者中,男性6例,女性5例;年龄32~79岁。8例无症状。CT显示,病灶均呈单发、实性,边界多较清,最大径为1.2~6.2 cm,CT值为31~78 HU,部分肿瘤周边可见长、短毛刺或浅分叶或胸膜牵拉;增强扫描显示,大部分病灶呈不均匀明显强化。免疫组化染色显示,CK5/6、p63均为阳性,6例PD-L1阳性表达。Epstein-Barr病毒编码小RNA(EBER)检查均为阳性。11例患者均接受了手术治疗,其中7例接受辅助化疗。截至2023年3月,11例患者均存活,10例患者无瘤生存,1例患者腹膜后淋巴结转移。结论PPLELC多为单发肿块,周围型为主,手术切除或手术联合化疗是首选治疗方案,患者预后较好。

Objective To investigate the imaging and pathological features of primary pulmonary lymphoepithelioma-like carcinoma(PPLELC),and expect to improve the understanding and diagnosis of this disease.Methods The imaging and clinical data of 11 cases with PPLELC confirmed by operation and pathology from January 2017 to December 2022 were retrospectively analyzed.Results This study consisted of 6 men and 5 women,aged 32-79 years.Eight patients were asymptomatic.CT scan showed most of tumors were solitary and solid with defined boundary,and CT value of them was about 31-79 HU.The sign of long,short burr,shallow lobes or pleural retraction could be seen around the tumor,and most lesions showed uneven and obvious enhancement on enhanced scan.Immunohistochemistry showed all patients were positive for CK5/6,p63,and 6 patients were positively presented for PD-L1.Epstein-Barr virus encoded small RNA test was positive in all patients.All patients underwent surgery.Among these patients,7 patients received adjuvant chemotherapy.Up to March 2023,11 patients were survived.Ten patients lived without tumors and 1 patient had retroperitoneal lymph node metastasis.Conclusion Most cases of PPLELC are single and peripheral masses.Surgical excision or surgical combination with chemotherapy is the first choice for PPLELC.The prognosis of patients is good.